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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (17)transporters (2) Show 19 proteins XML

enzymes (17)transporters (2) Show 19 proteins

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2024-05-19 04:28:27 UTC

HMDB ID

HMDB0000159

Secondary Accession Numbers

HMDB0000612
HMDB00159
HMDB00612

Metabolite Identification

Common Name

Phenylalanine

Description

Phenylalanine (Phe), also known as L-phenylalanine is an alpha-amino acid. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon). Amino acids are organic compounds that contain amino (-NH2) and carboxyl (-COOH) functional groups, along with a side chain (R group) specific to each amino acid. L-phenylalanine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Phenylalanine is found in all organisms ranging from bacteria to plants to animals. It is classified as an aromatic, non-polar amino acid. In humans, phenylalanine is an essential amino acid and the precursor of the amino acid tyrosine. Like tyrosine, phenylalanine is also a precursor for catecholamines including tyramine, dopamine, epinephrine, and norepinephrine. Catecholamines are neurotransmitters that act as adrenalin-like substances. Interestingly, several psychotropic drugs (mescaline, morphine, codeine, and papaverine) also have phenylalanine as a constituent. Phenylalanine is highly concentrated in the human brain and plasma. Normal metabolism of phenylalanine requires biopterin, iron, niacin, vitamin B6, copper, and vitamin C. An average adult ingests 5 g of phenylalanine per day and may optimally need up to 8 g daily. Phenylalanine is highly concentrated in a number of high protein foods, such as meat, cottage cheese, and wheat germ. An additional dietary source of phenylalanine is artificial sweeteners containing aspartame (a methyl ester of the aspartic acid/phenylalanine dipeptide). As a general rule, aspartame should be avoided by phenylketonurics and pregnant women. When present in sufficiently high levels (>500 uM), phenylalanine can act as a neurotoxin and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of phenylalanine are associated with at least five inborn errors of metabolism, including Hartnup disorder, hyperphenylalaninemia due to guanosine triphosphate cyclohydrolase deficiency, phenylketonuria (PKU), tyrosinemia type 2 (or Richner-Hanhart syndrome), and tyrosinemia type III (TYRO3). Phenylketonurics have elevated serum plasma levels of phenylalanine up to 400 times normal. High plasma concentrations of phenylalanine influence the blood-brain barrier transport of large neutral amino acids. The high plasma phenylalanine concentrations increase phenylalanine entry into the brain and restrict the entry of other large neutral amino acids (PMID: 19191004 ). Phenylalanine has been found to interfere with different cerebral enzyme systems. Untreated phenylketonuria (PKU) can lead to intellectual disability, seizures, behavioural problems, and mental disorders. It may also result in a musty smell and lighter skin. Classic PKU dramatically affects myelination and white matter tracts in untreated infants; this may be one major cause of neurological disorders associated with phenylketonuria. Mild phenylketonuria can act as an unsuspected cause of hyperactivity, learning problems, and other developmental problems in children. It has been recently suggested that PKU may resemble amyloid diseases, such as Alzheimer's disease and Parkinson's disease, due to the formation of toxic amyloid-like assemblies of phenylalanine (PMID: 22706200 ). Higher serum/plasma levels (>75 µmol/L) of phenylalanine are commonly seen in people suffering from heart failure, chronic inflammation or infections, as well those suffering from sepsis or septic shock (PMID: 31170548 ; PMID: 32618142 ). Normally most amino acid levels drop during inflammation or infection, so a rise in phenylalanine levels is considered unusual. While normal levels of phenylalanine are between 40-75 µmol/L, those in intensive care units with phenylalanine levels >110 µmol/L are at much greater risk of dying (PMID: 32618142 ). These moderately high levels of phenylalanine are not necessarily toxic, they are simply secondary indicators of something else that is seriously wrong. The metabolism of phenylalanine and its level in blood directly relates to the activity of the enzyme phenylalanine hydroxylase, which is functionally attenuated by high levels of inflammation. The attenuation of phenylalanine hydroxylase appears to be associated with insufficient tetrahydrobiopterin (BH4), the co‐factor for phenylalanine hydroxylase. In particular, inflammation‐induced production of reactive oxygen species (ROS) may consume a significant portion of BH4, thereby leaving phenylalanine unmetabolized. Pro‐inflammatory cytokine‐mediated inflammation also increases the production of neopterin at the expense of the production of BH4. Insufficient bioavailability of BH4 also leads to dysfunction in multiple systems, including nitric oxide synthase, tryptophan/kynurenine metabolism, the catecholamine pathway, the neural system, and thyroid hormone production (PMID: 32618142 ). High phenylalanine concentrations in septic patients may also reflect the breakdown of muscle tissues (leading to amino acid release) and the body’s differential metabolic capacity for different amino acids. Muscle tissue is easily able to oxidize branched chain amino acids to support its own energy requirements. Muscle tissue is also able to metabolize alanine, glycine, proline, aspartate, glutamate, histidine, glutamine and serine for gluconeogenesis, but aromatic amino acids such as phenylalanine and tyrosine as well as many cysteine-containing amino acids are not as easily metabolized. This may also account for the increase in the levels of phenylalanine and tyrosine seen during sepsis (PMID: 99098 ). Phenylalanine also has some potential benefits. Phenylalanine can act as an effective pain reliever. Its use in premenstrual syndrome and Parkinson's may enhance the effects of acupuncture and electric transcutaneous nerve stimulation (TENS). Phenylalanine and tyrosine, like L-DOPA, produce a catecholamine-like effect. Phenylalanine is better absorbed than tyrosine and may cause fewer headaches. Low phenylalanine diets have been prescribed for certain cancers with mixed results. For instance, some tumours use more phenylalanine than others (particularly melatonin-producing tumours called melanomas).Phenylalanine is elevated by a factor of two or more in the urine of patients with diet-controlled PKU or phenylketonuria (PMID: 37446577 ).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

HEADER    PROTEIN                                 27-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-SEP-20         0                                  
HETATM    1  C   UNK     0    8666.6948667.435   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8668.0288666.666   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8669.3598667.435   0.000  0.00  0.00           C+0
HETATM    4  O   UNK     0    8670.6938666.666   0.000  0.00  0.00           O+0
HETATM    5  O   UNK     0    8669.3598668.974   0.000  0.00  0.00           O+0
HETATM    6  N   UNK     0    8668.0288665.130   0.000  0.00  0.00           N+0
HETATM    7  C   UNK     0    8665.3638666.668   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0    8664.0298667.438   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0    8662.6968666.668   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0    8662.6968665.128   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0    8664.0298664.358   0.000  0.00  0.00           C+0
HETATM   12  C   UNK     0    8665.3638665.128   0.000  0.00  0.00           C+0
CONECT    1    2    7                                                       
CONECT    2    1    3    6                                                  
CONECT    3    2    4    5                                                  
CONECT    4    3                                                            
CONECT    5    3                                                            
CONECT    6    2                                                            
CONECT    7    8   12    1                                                  
CONECT    8    7    9                                                       
CONECT    9    8   10                                                       
CONECT   10    9   11                                                       
CONECT   11   10   12                                                       
CONECT   12   11    7                                                       
MASTER        0    0    0    0    0    0    0    0   12    0   24    0
END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(S)-2-Amino-3-phenylpropionic acid	ChEBI
(S)-alpha-Amino-beta-phenylpropionic acid	ChEBI
3-Phenyl-L-alanine	ChEBI
beta-Phenyl-L-alanine	ChEBI
F	ChEBI
Phe	ChEBI
PHENYLALANINE	ChEBI
(S)-2-Amino-3-phenylpropionate	Generator
(S)-a-Amino-b-phenylpropionate	Generator
(S)-a-Amino-b-phenylpropionic acid	Generator
(S)-alpha-Amino-beta-phenylpropionate	Generator
(S)-Α-amino-β-phenylpropionate	Generator
(S)-Α-amino-β-phenylpropionic acid	Generator
b-Phenyl-L-alanine	Generator
Β-phenyl-L-alanine	Generator
(-)-beta-Phenylalanine	HMDB
(L)-Phenylalanine	HMDB
(S)-(-)-Phenylalanine	HMDB
(S)-2-Amino-3-phenylpropanoate	HMDB
(S)-2-Amino-3-phenylpropanoic acid	HMDB
(S)-alpha-Amino-benzenepropanoate	HMDB
(S)-alpha-Amino-benzenepropanoic acid	HMDB
(S)-alpha-Aminobenzenepropanoate	HMDB
(S)-alpha-Aminobenzenepropanoic acid	HMDB
(S)-alpha-Aminohydrocinnamate	HMDB
(S)-alpha-Aminohydrocinnamic acid	HMDB
(S)-Phenylalanine	HMDB
alpha-Aminohydrocinnamate	HMDB
alpha-Aminohydrocinnamic acid	HMDB
beta-Phenyl-alpha-alanine	HMDB
beta-Phenylalanine	HMDB
L-2-Amino-3-phenylpropionate	HMDB
L-2-Amino-3-phenylpropionic acid	HMDB
Phenyl-alanine	HMDB
Phenylalamine	HMDB
L-Isomer phenylalanine	HMDB
Phenylalanine, L isomer	HMDB
Phenylalanine, L-isomer	HMDB
Endorphenyl	HMDB
(6S)-Tetrahydrofolate	HMDB
(6S)-Tetrahydrofolic acid	HMDB
(6S)-THFA	HMDB
5,6,7,8-Tetrahydrofolate	HMDB
Tetrahydrofolate	HMDB
THF	HMDB
5,6,7,8-Tetrahydrofolic acid	HMDB

Chemical Formula

C₉H₁₁NO₂

Average Molecular Weight

165.1891

Monoisotopic Molecular Weight

165.078978601

IUPAC Name

(2S)-2-amino-3-phenylpropanoic acid

Traditional Name

L-phenylalanine

CAS Registry Number

63-91-2

SMILES

N[C@@H](CC1=CC=CC=C1)C(O)=O

InChI Identifier

InChI=1S/C9H11NO2/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5,8H,6,10H2,(H,11,12)/t8-/m0/s1

InChI Key

COLNVLDHVKWLRT-QMMMGPOBSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as phenylalanine and derivatives. Phenylalanine and derivatives are compounds containing phenylalanine or a derivative thereof resulting from reaction of phenylalanine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Phenylalanine and derivatives

Alternative Parents

Substituents

Phenylalanine or derivatives
3-phenylpropanoic-acid
Alpha-amino acid
Amphetamine or derivatives
L-alpha-amino acid
Aralkylamine
Monocyclic benzene moiety
Benzenoid
Amino acid
Carboxylic acid
Monocarboxylic acid or derivatives
Organic nitrogen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Hydrocarbon derivative
Primary aliphatic amine
Organic oxide
Carbonyl group
Organopnictogen compound
Organic oxygen compound
Amine
Aromatic homomonocyclic compound

Molecular Framework

Aromatic homomonocyclic compounds

External Descriptors

proteinogenic amino acid (CHEBI:17295 )
L-alpha-amino acid (CHEBI:17295 )
phenylalanine (CHEBI:17295 )
erythrose 4-phosphate/phosphoenolpyruvate family amino acid (CHEBI:17295 )
Common amino acids (C00079 )

Ontology

Not Available

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	283 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	26.9 mg/mL	Not Available
LogP	-1.38	AVDEEF,A (1997)

Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct Type	Data Source	CCS Value (Å²)	Reference
[M-H]-	Baker	140.95	30932474
[M-H]-	McLean	141.345	30932474
[M-H]-	MetCCS_test_neg	137.0	30932474
[M+H]+	Astarita_pos	135.0	30932474
[M+H]+	Baker	141.02	30932474
[M+H]+	McLean	141.289	30932474
[M+H]+	MetCCS_test_pos	140.288	30932474
[M-H]-	Not Available	140.954	http://allccs.zhulab.cn/database/detail?ID=AllCCS00001752

Predicted Molecular Properties

Property	Value	Source
Water Solubility	4.14 g/L	ALOGPS
logP	-1.4	ALOGPS
logP	-1.2	ChemAxon
logS	-1.6	ALOGPS
pKa (Strongest Acidic)	2.47	ChemAxon
pKa (Strongest Basic)	9.45	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	63.32 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	45.12 m³·mol⁻¹	ChemAxon
Polarizability	17.03 Å³	ChemAxon
Number of Rings	1	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	136.759	31661259
DarkChem	[M-H]-	134.973	31661259
AllCCS	[M+H]+	136.853	32859911
AllCCS	[M-H]-	135.247	32859911
DeepCCS	[M+H]+	133.926	30932474
DeepCCS	[M+H]+	130.072	30932474
DeepCCS	[M-H]-	130.506	30932474
DeepCCS	[M-H]-	126.243	30932474
DeepCCS	[M-2H]-	167.718	30932474
DeepCCS	[M+Na]+	143.256	30932474
DeepCCS	[M-2H]-	163.625	30932474
DeepCCS	[M+Na]+	139.115	30932474
AllCCS	[M+H]+	136.9	32859911
AllCCS	[M+H-H2O]+	132.5	32859911
AllCCS	[M+NH4]+	140.9	32859911
AllCCS	[M+Na]+	142.0	32859911
AllCCS	[M-H]-	135.2	32859911
AllCCS	[M+Na-2H]-	136.3	32859911
AllCCS	[M+HCOO]-	137.6	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
L-Phenylalanine	N[C@@H](CC1=CC=CC=C1)C(O)=O	2751.8	Standard polar	33892256
L-Phenylalanine	N[C@@H](CC1=CC=CC=C1)C(O)=O	1455.3	Standard non polar	33892256
L-Phenylalanine	N[C@@H](CC1=CC=CC=C1)C(O)=O	1759.2	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
L-Phenylalanine,1TMS,isomer #1	C[Si](C)(C)OC(=O)[C@@H](N)CC1=CC=CC=C1	1560.0	Semi standard non polar	33892256
L-Phenylalanine,1TMS,isomer #2	C[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O	1640.3	Semi standard non polar	33892256
L-Phenylalanine,2TMS,isomer #1	C[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O[Si](C)(C)C	1633.5	Semi standard non polar	33892256
L-Phenylalanine,2TMS,isomer #1	C[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O[Si](C)(C)C	1685.3	Standard non polar	33892256
L-Phenylalanine,2TMS,isomer #1	C[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O[Si](C)(C)C	1959.2	Standard polar	33892256
L-Phenylalanine,2TMS,isomer #2	C[Si](C)(C)N([C@@H](CC1=CC=CC=C1)C(=O)O)[Si](C)(C)C	1794.9	Semi standard non polar	33892256
L-Phenylalanine,2TMS,isomer #2	C[Si](C)(C)N([C@@H](CC1=CC=CC=C1)C(=O)O)[Si](C)(C)C	1745.4	Standard non polar	33892256
L-Phenylalanine,2TMS,isomer #2	C[Si](C)(C)N([C@@H](CC1=CC=CC=C1)C(=O)O)[Si](C)(C)C	2109.2	Standard polar	33892256
L-Phenylalanine,3TMS,isomer #1	C[Si](C)(C)OC(=O)[C@H](CC1=CC=CC=C1)N([Si](C)(C)C)[Si](C)(C)C	1829.0	Semi standard non polar	33892256
L-Phenylalanine,3TMS,isomer #1	C[Si](C)(C)OC(=O)[C@H](CC1=CC=CC=C1)N([Si](C)(C)C)[Si](C)(C)C	1803.3	Standard non polar	33892256
L-Phenylalanine,3TMS,isomer #1	C[Si](C)(C)OC(=O)[C@H](CC1=CC=CC=C1)N([Si](C)(C)C)[Si](C)(C)C	1930.1	Standard polar	33892256
L-Phenylalanine,1TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CC1=CC=CC=C1	1804.5	Semi standard non polar	33892256
L-Phenylalanine,1TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O	1869.4	Semi standard non polar	33892256
L-Phenylalanine,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O[Si](C)(C)C(C)(C)C	2084.8	Semi standard non polar	33892256
L-Phenylalanine,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O[Si](C)(C)C(C)(C)C	2095.0	Standard non polar	33892256
L-Phenylalanine,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N[C@@H](CC1=CC=CC=C1)C(=O)O[Si](C)(C)C(C)(C)C	2236.3	Standard polar	33892256
L-Phenylalanine,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N([C@@H](CC1=CC=CC=C1)C(=O)O)[Si](C)(C)C(C)(C)C	2238.1	Semi standard non polar	33892256
L-Phenylalanine,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N([C@@H](CC1=CC=CC=C1)C(=O)O)[Si](C)(C)C(C)(C)C	2161.9	Standard non polar	33892256
L-Phenylalanine,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N([C@@H](CC1=CC=CC=C1)C(=O)O)[Si](C)(C)C(C)(C)C	2295.2	Standard polar	33892256
L-Phenylalanine,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@H](CC1=CC=CC=C1)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2497.1	Semi standard non polar	33892256
L-Phenylalanine,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@H](CC1=CC=CC=C1)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2402.1	Standard non polar	33892256
L-Phenylalanine,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@H](CC1=CC=CC=C1)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2284.9	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0fr6-1930000000-a37fbccaf826443ef70c	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-014l-1940000000-537e2725d621246630c1	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-00di-9630000000-ead0919f9a19d2352d80	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-MS (1 TMS)	splash10-00di-2900000000-cb2d6dc4bf9515150328	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-MS (2 TMS)	splash10-014l-2960000000-f77489792f0652dd5613	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine EI-B (Non-derivatized)	splash10-014l-0970000000-792b341dd28b9e30bac2	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-EI-TOF (Non-derivatized)	splash10-0fr6-1930000000-a37fbccaf826443ef70c	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-EI-TOF (Non-derivatized)	splash10-014l-1940000000-537e2725d621246630c1	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-EI-QQ (Non-derivatized)	splash10-0ul9-3659000000-b85be4e71798e2fdc47b	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-EI-TOF (Non-derivatized)	splash10-00di-9630000000-ead0919f9a19d2352d80	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-MS (Non-derivatized)	splash10-00di-2900000000-cb2d6dc4bf9515150328	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Phenylalanine GC-MS (Non-derivatized)	splash10-014l-2960000000-f77489792f0652dd5613	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Phenylalanine GC-MS (Non-derivatized) - 70eV, Positive	splash10-006x-9600000000-df38fcb743d8f44fb876	2016-09-22	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Phenylalanine GC-MS (1 TMS) - 70eV, Positive	splash10-00di-7900000000-f21569d2ec75b88e1bda	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Phenylalanine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Phenylalanine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Phenylalanine GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Phenylalanine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Phenylalanine GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-00dl-9300000000-4782928378caea601f9b	2018-05-25	Not Available	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-014i-0900000000-0f3b994108b8a9fd2a56	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-0gb9-2900000000-c14d44c8a67621757f3d	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-004i-9300000000-08c642dab7f49c00da43	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-0900000000-7dce1e473976f7d2143e	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-0f6x-9600000000-711557391093b0d8500a	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-00di-0900000000-0c25a5c116eac7bb059b	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-00di-0900000000-2804f79084ac4e67e155	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-0900000000-8a71bb1f8424064d7caf	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-0f6x-9800000000-e027ff6bb67ce55e80a5	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-0900000000-9f6185e9c7d54189f369	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-00di-0900000000-df5f72fe2bba91742427	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-0ir3-0988735721-bac229222fe7b52812a8	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-0002-0900000000-453477dec847a3672ffe	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-0a4i-0010963000-079a66bf710f6778bceb	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-00di-0000009000-62dbe98de4ecde484fb3	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOF	splash10-03di-0900000000-80558c17dc1845663c85	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOF	splash10-0002-3900000000-e1ee31d41e48824e84b7	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOF	splash10-0f6x-9500000000-cc11290a37615f24e16e	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOF	splash10-0006-9000000000-1a6020bce0e1a9a14832	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOF	splash10-0006-9000000000-38c044a112152626962e	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive-QTOF	splash10-014i-0900000000-9b908abfcb63153d60b3	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive-QTOF	splash10-00di-1900000000-3ba5964e151bb1d56188	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive-QTOF	splash10-00di-2900000000-20a7d24da0281f5b3b78	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive-QTOF	splash10-0udi-5900000000-1800642a835b49f3398a	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Phenylalanine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive-QTOF	splash10-0fb9-9300000000-f262384c85fb843f8a11	2012-08-31	HMDB team, MONA	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Experimental 1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, experimental)	2012-12-04	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Experimental 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, experimental)	2021-10-10	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2012-12-05	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Quadrupole Ion Trap, ESI+, Adduct: [M+H]+)	2022-02-11	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Quadrupole Ion Trap, ESI-, Adduct: [M-H]-)	2022-02-11	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)	2023-02-03	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Mitochondria

Biospecimen Locations

Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

All Tissues
Placenta
Prostate

Pathways

Name	SMPDB/PathBank	KEGG
Transcription/Translation	SMP00019	Not Available
Phenylalanine and Tyrosine Metabolism
Phenylketonuria		Not Available
Tyrosinemia Type 2 (or Richner-Hanhart syndrome)		Not Available
Tyrosinemia Type 3 (TYRO3)		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	78.1 +/- 20.5 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	48 (50-52) uM	Adult (>18 years old)	Both	Normal	822705	details
Blood	Detected and Quantified	85.2 +/- 23 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	57.0 (48.0-66.0) uM	Adult (>18 years old)	Both	Normal	12297216	details
Blood	Detected and Quantified	16-71 uM	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	24-80 uM	Infant (1 - 3 months old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	34-101 uM	Children (3 months - 6 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	39-76 uM	Children (6 - 18 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	80.0 +/- 20.0 uM	Newborn (0-30 days old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	60.0 +/- 8.0 uM	Children (1-13 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	65.0 +/- 9.0 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	56.0 +/- 8.0 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	<70 uM	Newborn (0-30 days old)	Not Specified	Normal	8178819	details
Blood	Detected and Quantified	108 (96.80-124) uM	Newborn (0-30 days old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	93.6 (75.8-114) uM	Infant (0-1 year old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	70(10.98) uM	Adult (>18 years old)	Both	Normal	26010610	details
Blood	Detected and Quantified	60 +/- 7 uM	Adult (>18 years old)	Male	Normal	2415198	details
Blood	Detected and Quantified	52 +/- 5 uM	Adult (>18 years old)	Female	Normal	2415198	details
Blood	Detected and Quantified	47 +/- 5 uM	Children (1 - 13 years old)	Both	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	40.0-75.0 uM	Adult (>18 years old)	Both	Normal	The Metabolo...	details
Blood	Detected and Quantified	56 +/- 8 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	60.0 +/- 7.75 uM	Adult (>18 years old)	Both	Normal	28278231	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	20143319	details
Blood	Detected and Quantified	65 +/- 9 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	81.9(70.6-94.3) uM	Children (1-13 uears old)	Both	Normal	27294788	details
Blood	Detected and Quantified	<120 uM	Newborn (0-30 days old)	Not Specified	Normal	3873535	details
Blood	Detected and Quantified	166.0 +/- 3.0 uM	Adult (>18 years old)	Both	Normal	16789974	details
Breast Milk	Detected and Quantified	13.1 +/- 3.9 uM	Adult (>18 years old)	Female	Normal	24027187	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	10.4 (7.58-13.2) uM	Adult (>18 years old)	Both	Normal	12834252	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	15 +/- 13 uM	Adult (>18 years old)	Both	Normal	18502700	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	18.0 +/- 7.2 uM	Adult (>18 years old)	Not Specified	Normal	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	12.5 +/- 2.9 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	10.8 +/- 4.2 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	12.5 +/- 2.6 uM	Adult (>18 years old)	Not Specified	Normal	22546835	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.3 +/- 0.81 uM	Adult (>18 years old)	Both	Normal	7595563	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	6.5 +/- 1.2 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4-20 uM	Children (0 - 10 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	6.6-15 uM	Adolescent (>11 years old)	Both	Normal	BC Children's Hos...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	23940645	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Normal	24130822	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	Mette S. Sch...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25670064	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	22411374	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Both	Normal	25486321	details
Feces	Detected and Quantified	3450.591 (2159.142-3652.380) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected and Quantified	3813.811 (665.904-6961.718) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected and Quantified	6396.709 (3632.201-9161.218) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	22944170	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24029555	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18052033	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	25985090	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected and Quantified	41.770 +/- 42.134 nmol/g wet feces	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	24375085	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	26709303	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28842642	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27065191	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18085514	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected and Quantified	253.0433 +/- 397.726 nmol/g wet feces	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected and Quantified	670 +/- 410 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28587349	details
Feces	Detected and Quantified	120 +/- 120 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25598765	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	28270806	details
Saliva	Detected and Quantified	10.69 +/- 6.28 uM	Adult (>18 years old)	Female	Normal	23857558	details
Saliva	Detected and Quantified	5.55 +/- 4.30 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	<1-68 uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	<1-54 uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	18 (<1-112) uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	8 (<1-54) uM	Adult (>18 years old)	Female	Normal	19259987	details
Saliva	Detected and Quantified	8.24 +/- 6.51 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21190195	details
Saliva	Detected and Quantified	10.8 +/- 3.3 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	1-187 uM	Adult (>18 years old)	Male	normal	19259987	details
Saliva	Detected and Quantified	1-32 uM	Adult (>18 years old)	Male	normal	19259987	details
Saliva	Detected and Quantified	12.0 +/- 6.5 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	7.1 +/- 6.4 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	43.70 +/- 23.30 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected and Quantified	10.3 +/- 3.8 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	>10 uM	Adult (>18 years old)	Both	Normal	12097436	details
Sweat	Detected and Quantified	40-260 uM	Adult (60 years old)	Male	Normal	22194922	details
Sweat	Detected and Quantified	< 10 uM	Adult (40 years old)	Male	Normal	22194922	details
Sweat	Detected but not Quantified	Not Quantified	Adult	Both	Normal	26755145	details
Urine	Detected and Quantified	3.17 - 37.65 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	6.90 - 32.79 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	11.76 - 32.79 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	10.51 - 36.41 umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	6.90 - 24.19 umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	4.41 - 30.30 umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0.79 - 21.71 umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0.79 - 21.71 umol/mmol creatinine	Adult (>21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	7.8 (5.0-11.3) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	2.38-11.99 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	5.9 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details
Urine	Detected and Quantified	1.24-12.56 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	14.389 +/- 18.635 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	3.63-18.17 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	David F. Putnam C...	details
Urine	Detected and Quantified	2.6-4.4 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	0.40-21.24 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	25536744	details
Urine	Detected and Quantified	13.02 +/- 9.04 umol/mmol creatinine	Children (0-2 years old)	Male	Normal	25536744	details
Urine	Detected and Quantified	13.79 +/- 9.45 umol/mmol creatinine	Children (0-2 years old)	Female	Normal	25536744	details
Urine	Detected and Quantified	3.65 +/- 2.73 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	25536744	details
Urine	Detected and Quantified	8.04 +/- 6.10 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	25536744	details
Urine	Detected and Quantified	1.47-7.92 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	6.4 (3.5-11.2) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	0.7 +/- 0.3 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	3168222	details
Urine	Detected and Quantified	1.1 +/- 0.5 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	3168222	details
Urine	Detected and Quantified	1.5 +/- 0.6 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	3168222	details
Urine	Detected and Quantified	1.58-31.67 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	4.605 (1.645-7.566) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Not Available	details
Urine	Detected and Quantified	4.50 +/- 1.15 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	5.9 (2.8-10.8) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	14.67 (5.33-24) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	1.61 +/- 1.0 umol/mmol creatinine	Children (1-13 years old)	Female	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	4.5 +/- 1.87 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	4.0 +/- 1.76 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	3.85-28.73 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	4-30 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	30830347	details
Urine	Detected and Quantified	4-30 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	30830347	details
Urine	Detected and Quantified	3.1-5.0 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	12.44 +/- 9.04 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	18.09 +/- 15.83 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	26.004 +/- 13.57 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	3.7 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details
Urine	Detected and Quantified	23.59 +/- 16.02 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected and Quantified	4.14-14.15 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	15.2043 +/- 4.9763 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	7.59 +/- 2.54 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	7.57 +/- 2.52 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	32340350	details
Urine	Detected and Quantified	2.26-16.97 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Urine	Detected and Quantified	9.18 (7.09-15.78) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	60.3 +/- 16.8 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	66.5 +/- 14.6 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	49.6 (47.1-52.1) uM	Adult (>18 years old)	Both	Epilepsy	14992292	details
Blood	Detected and Quantified	53.1 (50.8-55.4) uM	Children (1-13 years old)	Both	Epilepsy	14992292	details
Blood	Detected and Quantified	75.0 (67.0-83.0) uM	Adult (>18 years old)	Both	Dengue fever	11237944	details
Blood	Detected and Quantified	101.0 (97.0-105.0) uM	Adult (>18 years old)	Both	Myocardial infarction	822705	details
Blood	Detected and Quantified	98.0 - 113.0 uM	Newborn (0-30 days old)	Both	Maple syrup urine disease (MSUD)	12101068	details
Blood	Detected and Quantified	143.0 (128.0-158.0) uM	Adult (>18 years old)	Both	Bacterial infections (assorted)	822705	details
Blood	Detected and Quantified	171.0 (140.0-202.0) uM	Adult (>18 years old)	Both	Viral infections (assorted)	822705	details
Blood	Detected and Quantified	571.0 - 1023.0 uM	Newborn (0-30 days old)	Both	Phenylketonuria	12101068	details
Blood	Detected and Quantified	44.8 +/- 21 uM	Adult (>18 years old)	Both	Heart Transplant	21359215	details
Blood	Detected and Quantified	74.91 +/- 34.67 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	58.24 +/- 28.67 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected and Quantified	62.9 (12.1) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	79.6 (42.7) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Blood	Detected and Quantified	360-1200 uM	Newborn (0-30 days old)	Both	Hyperphenylalaninemia due to 6-pyruvoyltetrahydropterin synthase deficiency (PTPS)	8178819	details
Blood	Detected and Quantified	152(100-450) uM	Adult (>18 years old)	Both	Sepsis	26847922	details
Blood	Detected and Quantified	44.46 (12.81) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	1210.734 uM	Infant (0-1 year old)	Male	Phenylketonuria	15168722	details
Blood	Detected and Quantified	43.9 (15.78) uM	Adult (>18 years old)	Female	Pregnancy	24704061	details
Blood	Detected and Quantified	1004.909-1210.734 uM	Children (1 - 13 years old)	Male	Phenylketonuria	15168722	details
Blood	Detected and Quantified	78.0 (45.9) uM	Adult (>18 years old)	Female	Late-onset preeclampsia	23159745	details
Blood	Detected and Quantified	76.83(15.96) uM	Adult (>18 years old)	Both	Heart failure with preserved ejection fraction	26010610	details
Blood	Detected and Quantified	80.9 (45.4) uM	Adult (>18 years old)	Female	Pregnancy	23159745	details
Blood	Detected and Quantified	64 +/- 8 uM	Adult (>18 years old)	Male	Schizophrenia	2415198	details
Blood	Detected and Quantified	57 +/- 8 uM	Adolescent (13-18 years old)	Female	Schizophrenia	2415198	details
Blood	Detected and Quantified	39.954 uM	Newborn (0-30 days old)	Female	Hyperphenylalaninemia due to pterin-4a-carbinolamine dehydratase	3200274	details
Blood	Detected and Quantified	85.5(75.5-99.5) uM	Children (1-13 uears old)	Both	Environmental enteric dysfunction	27294788	details
Blood	Detected and Quantified	71.77 +/- 9.21 uM	Elderly (>65 years old)	Both	Alzheimer's disease	17031479	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Pancreatic ductal adenocarcinoma	20143319	details
Blood	Detected and Quantified	1000-2400 uM	Newborn (0-30 days old)	Male	Hyperphenylalaninemia due to guanosine triphosphate cyclohydrolase deficiency	3873535	details
Blood	Detected and Quantified	55.4 (27.0) uM	Adult (>18 years old)	Female	Down syndrome pregnancy	23313728	details
Blood	Detected and Quantified	46.2 (30.8) uM	Adult (>18 years old)	Female	Pregnancy	23313728	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	14.7 (11.6-17.8) uM	Adult (>18 years old)	Both	Hypothyroidism	9849813	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	18.6 +/- 5.2 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	16.4 +/- 6.3 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	14.7 +/- 3.1 uM	Adult (>18 years old)	Not Specified	Hypothyroidism	9849813	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	6.0 +/- 0.8 uM	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	14.7 +/- 3.1 uM	Adult (>18 years old)	Both	Hypothyroidism	9849813	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.6 +/- 1.0 uM	Adult (>18 years old)	Both	Schizophrenia	7595563	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Autism	24130822	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	ankylosing spondylitis	Tie-juan Sha...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	rheumatoid arthritis	Tie-juan Sha...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ileal Crohn's disease	19636438	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Cryptosporidium infection	22944170	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Myalgic encephalomyelitis/chronic fatigue syndrome	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable bowel syndrome	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohn disease	28842642	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	28587349	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	28842642	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable Bowel Syndrome	21494186	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohns disease	25598765	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	25598765	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Gout	28270806	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Attachment loss	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Periodontal Probing Depth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Breast cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Pancreatic cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Periodontal diseases	20300169	details
Saliva	Detected and Quantified	5.97 +/- 0.09 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral squamous cell carcinoma (OSCC)	21190195	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral leukoplakia (OLK)	21190195	details
Saliva	Detected and Quantified	9.01 +/- 2.41 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected and Quantified	8.55 +/- 1.22 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Bladder cancer	28157703	details
Urine	Detected and Quantified	17.479 +/- 13.832 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	253.238 umol/mmol creatinine	Children (1 - 13 years old)	Male	Phenylketonuria	15168722	details
Urine	Detected and Quantified	414.763 umol/mmol creatinine	Infant (0-1 year old)	Male	Phenylketonuria	15168722	details
Urine	Detected and Quantified	1.37 +/- 0.18 umol/mmol creatinine	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Urine	Detected and Quantified	11.9892 +/- 6.1922 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	10.2356 +/- 6.7416 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details

Associated Disorders and Diseases

Disease References

Epilepsy
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Dengue fever
Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed:11237944 ]
Myocardial infarction
Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
Maple syrup urine disease
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
Bacterial infections
Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
Viral infection
Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
Phenylketonuria
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ] Swarna M, Jyothy A, Usha Rani P, Reddy PP: Amino acid disorders in mental retardation: a two-decade study from Andhra Pradesh. Biochem Genet. 2004 Apr;42(3-4):85-98. [PubMed:15168722 ]
Alzheimer's disease
Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ] Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Early preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Pregnancy
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ] Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ] Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ] Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ] Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Late-onset preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
Obesity
Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
Pterin-4a carbinolamine dehydratase deficiency
Blaskovics M, Giudici TA: A new variant of biopterin deficiency. N Engl J Med. 1988 Dec 15;319(24):1611-2. doi: 10.1056/NEJM198812153192420. [PubMed:3200274 ]
Guanosine triphosphate cyclohydrolase deficiency
Dhondt JL, Farriaux JP, Boudha A, Largilliere C, Ringel J, Roger MM, Leeming RJ: Neonatal hyperphenylalaninemia presumably caused by guanosine triphosphate-cyclohydrolase deficiency. J Pediatr. 1985 Jun;106(6):954-6. [PubMed:3873535 ]
6-Pyruvoyltetrahydropterin synthase deficiency
Thony B, Leimbacher W, Blau N, Harvie A, Heizmann CW: Hyperphenylalaninemia due to defects in tetrahydrobiopterin metabolism: molecular characterization of mutations in 6-pyruvoyl-tetrahydropterin synthase. Am J Hum Genet. 1994 May;54(5):782-92. [PubMed:8178819 ]
Sepsis
Ferrario M, Cambiaghi A, Brunelli L, Giordano S, Caironi P, Guatteri L, Raimondi F, Gattinoni L, Latini R, Masson S, Ristagno G, Pastorelli R: Mortality prediction in patients with severe septic shock: a pilot study using a target metabolomics approach. Sci Rep. 2016 Feb 5;6:20391. doi: 10.1038/srep20391. [PubMed:26847922 ]
Hypothyroidism
Sjoberg S, Eriksson M, Nordin C: L-thyroxine treatment and neurotransmitter levels in the cerebrospinal fluid of hypothyroid patients: a pilot study. Eur J Endocrinol. 1998 Nov;139(5):493-7. [PubMed:9849813 ]
Leukemia
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Schizophrenia
Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [PubMed:7595563 ] Bjerkenstedt L, Edman G, Hagenfeldt L, Sedvall G, Wiesel FA: Plasma amino acids in relation to cerebrospinal fluid monoamine metabolites in schizophrenic patients and healthy controls. Br J Psychiatry. 1985 Sep;147:276-82. [PubMed:2415198 ]
Irritable bowel syndrome
Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] Hong YS, Hong KS, Park MH, Ahn YT, Lee JH, Huh CS, Lee J, Kim IK, Hwang GS, Kim JS: Metabonomic understanding of probiotic effects in humans with irritable bowel syndrome. J Clin Gastroenterol. 2011 May-Jun;45(5):415-25. doi: 10.1097/MCG.0b013e318207f76c. [PubMed:21494186 ]
Ulcerative colitis
Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ] Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Colorectal cancer
Weir TL, Manter DK, Sheflin AM, Barnett BA, Heuberger AL, Ryan EP: Stool microbiome and metabolome differences between colorectal cancer patients and healthy adults. PLoS One. 2013 Aug 6;8(8):e70803. doi: 10.1371/journal.pone.0070803. Print 2013. [PubMed:23940645 ] Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ] Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ] Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ] Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ] Wang X, Wang J, Rao B, Deng L: Gut flora profiling and fecal metabolite composition of colorectal cancer patients and healthy individuals. Exp Ther Med. 2017 Jun;13(6):2848-2854. doi: 10.3892/etm.2017.4367. Epub 2017 Apr 20. [PubMed:28587349 ]
Autism
De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
Crohn's disease
Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ] Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Gout
Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
Rheumatoid arthritis
Tie-juan ShaoZhi-xing HeZhi-jun XieHai-chang LiMei-jiao WangCheng-ping Wen. Characterization of ankylosing spondylitis and rheumatoid arthritis using 1H NMR-based metabolomics of human fecal extracts. Metabolomics. April 2016, 12:70 [Link]
Perillyl alcohol administration for cancer treatment
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Pancreatic cancer
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Periodontal disease
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Frontotemporal dementia
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Attachment loss
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.

Associated OMIM IDs

248600 (Maple syrup urine disease)
261600 (Phenylketonuria)
104300 (Alzheimer's disease)
601665 (Obesity)
264070 (Pterin-4a carbinolamine dehydratase deficiency)
233910 (Guanosine triphosphate cyclohydrolase deficiency)
261640 (6-Pyruvoyltetrahydropterin synthase deficiency)
181500 (Schizophrenia)
114500 (Colorectal cancer)
209850 (Autism)
266600 (Crohn's disease)
138900 (Gout)
180300 (Rheumatoid arthritis)
260350 (Pancreatic cancer)
170650 (Periodontal disease)
600274 (Frontotemporal dementia)
610247 (Eosinophilic esophagitis)

External Links

DrugBank ID

DB00120

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

6140

PDB ID

Not Available

ChEBI ID

17295

Food Biomarker Ontology

Not Available

VMH ID

PHE_L

MarkerDB ID

MDB00000077

Good Scents ID

Not Available

References

Synthesis Reference

Zhou, Hua; Zhong, Yao; Sun, Guanghai; Wei, Ping. Preparation of L-phenylalanine by an aqueous two-phase system. Huaxue Fanying Gongcheng Yu Gongyi (2006), 22(2), 146-150.

Material Safety Data Sheet (MSDS)

Not Available

General References

Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
Sjoberg S, Eriksson M, Nordin C: L-thyroxine treatment and neurotransmitter levels in the cerebrospinal fluid of hypothyroid patients: a pilot study. Eur J Endocrinol. 1998 Nov;139(5):493-7. [PubMed:9849813 ]
Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [PubMed:6198473 ]
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
Doellgast GJ, Meis PJ: Use of specific inhibitors to disciminate alkaline phosphatase isoenzymes originating from human liver, placenta and intestine: absence of meconial alkaline phosphatase in maternal serum. Clin Chem. 1979 Jul;25(7):1230-3. [PubMed:455643 ]
Kersemans V, Cornelissen B, Kersemans K, Bauwens M, Achten E, Dierckx RA, Mertens J, Slegers G: In vivo characterization of 123/125I-2-iodo-L-phenylalanine in an R1M rhabdomyosarcoma athymic mouse model as a potential tumor tracer for SPECT. J Nucl Med. 2005 Mar;46(3):532-9. [PubMed:15750170 ]
Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed:11237944 ]
van Spronsen FJ, Hoeksma M, Reijngoud DJ: Brain dysfunction in phenylketonuria: is phenylalanine toxicity the only possible cause? J Inherit Metab Dis. 2009 Feb;32(1):46-51. doi: 10.1007/s10545-008-0946-2. Epub 2009 Jan 13. [PubMed:19191004 ]
Adler-Abramovich L, Vaks L, Carny O, Trudler D, Magno A, Caflisch A, Frenkel D, Gazit E: Phenylalanine assembly into toxic fibrils suggests amyloid etiology in phenylketonuria. Nat Chem Biol. 2012 Aug;8(8):701-6. doi: 10.1038/nchembio.1002. Epub 2012 Jun 17. [PubMed:22706200 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]
Huang SS, Lin JY, Chen WS, Liu MH, Cheng CW, Cheng ML, Wang CH: Phenylalanine- and leucine-defined metabolic types identify high mortality risk in patients with severe infection. Int J Infect Dis. 2019 Aug;85:143-149. doi: 10.1016/j.ijid.2019.05.030. Epub 2019 Jun 3. [PubMed:31170548 ]
Chen WS, Wang CH, Cheng CW, Liu MH, Chu CM, Wu HP, Huang PC, Lin YT, Ko T, Chen WH, Wang HJ, Lee SC, Liang CY: Elevated plasma phenylalanine predicts mortality in critical patients with heart failure. ESC Heart Fail. 2020 Oct;7(5):2884-2893. doi: 10.1002/ehf2.12896. Epub 2020 Jul 2. [PubMed:32618142 ]
Freund HR, Ryan JA Jr, Fischer JE: Amino acid derangements in patients with sepsis: treatment with branched chain amino acid rich infusions. Ann Surg. 1978 Sep;188(3):423-30. doi: 10.1097/00000658-197809000-00017. [PubMed:99098 ]
Knuplez E, Marsche G: An Updated Review of Pro- and Anti-Inflammatory Properties of Plasma Lysophosphatidylcholines in the Vascular System. Int J Mol Sci. 2020 Jun 24;21(12). pii: ijms21124501. doi: 10.3390/ijms21124501. [PubMed:32599910 ]
Cannet C, Bayat A, Frauendienst-Egger G, Freisinger P, Spraul M, Himmelreich N, Kockaya M, Ahring K, Godejohann M, MacDonald A, Trefz F: Phenylketonuria (PKU) Urinary Metabolomic Phenotype Is Defined by Genotype and Metabolite Imbalance: Results in 51 Early Treated Patients Using Ex Vivo (1)H-NMR Analysis. Molecules. 2023 Jun 22;28(13):4916. doi: 10.3390/molecules28134916. [PubMed:37446577 ]

Only showing the first 10 proteins. There are 19 proteins in total.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. Tyrosine 3-monooxygenase

General function:: Involved in monooxygenase activity
Specific function:: Plays an important role in the physiology of adrenergic neurons.
Gene Name:: TH
Uniprot ID:: P07101
Molecular weight:: 55611.26

References

Fukami MH, Haavik J, Flatmark T: Phenylalanine as substrate for tyrosine hydroxylase in bovine adrenal chromaffin cells. Biochem J. 1990 Jun 1;268(2):525-8. [PubMed:1973034 ]
Ogawa S, Ichinose H: Effect of metals and phenylalanine on the activity of human tryptophan hydroxylase-2: comparison with that on tyrosine hydroxylase activity. Neurosci Lett. 2006 Jul 3;401(3):261-5. Epub 2006 Apr 11. [PubMed:16581181 ]
Schallreuter KU, Kothari S, Hasse S, Kauser S, Lindsey NJ, Gibbons NC, Hibberts N, Wood JM: In situ and in vitro evidence for DCoH/HNF-1 alpha transcription of tyrosinase in human skin melanocytes. Biochem Biophys Res Commun. 2003 Feb 7;301(2):610-6. [PubMed:12565907 ]
McQuade PS, Juorio AV: The effect of various amino acids and drugs on the para- and meta-hydroxyphenylacetic acid concentrations in the mouse caudate nucleus. Neurochem Res. 1983 Jul;8(7):903-12. [PubMed:6621777 ]
Fusetti F, Erlandsen H, Flatmark T, Stevens RC: Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria. J Biol Chem. 1998 Jul 3;273(27):16962-7. [PubMed:9642259 ]
Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]

Enzyme Details

2. Phenylalanine--tRNA ligase alpha subunit

General function:: Involved in nucleotide binding
Specific function:: Not Available
Gene Name:: FARSA
Uniprot ID:: Q9Y285
Molecular weight:: 57563.225

Reactions

Adenosine triphosphate + Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-phenylalanyl-tRNA(Phe)	details
Adenosine triphosphate + Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-Phenylalanyl-tRNA(Phe)	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Kodama K, Fukuzawa S, Sakamoto K, Nakayama H, Kigawa T, Yabuki T, Matsuda N, Shirouzu M, Takio K, Tachibana K, Yokoyama S: A new protein engineering approach combining chemistry and biology, part I; site-specific incorporation of 4-iodo-L-phenylalanine in vitro by using misacylated suppressor tRNAPhe. Chembiochem. 2006 Oct;7(10):1577-81. [PubMed:16969782 ]
Kotik-Kogan O, Moor N, Tworowski D, Safro M: Structural basis for discrimination of L-phenylalanine from L-tyrosine by phenylalanyl-tRNA synthetase. Structure. 2005 Dec;13(12):1799-807. [PubMed:16338408 ]

Enzyme Details

3. Phenylalanine--tRNA ligase, mitochondrial

General function:: Involved in nucleotide binding
Specific function:: Catalyzes direct attachment of p-Tyr (Tyr) to tRNAPhe. Permits also, with a lower efficiency, the attachment of m-Tyr to tRNAPhe, thereby opening the way for delivery of the misacylated tRNA to the ribosome and incorporation of ROS-damaged amino acid into proteins.
Gene Name:: FARS2
Uniprot ID:: O95363
Molecular weight:: 52356.21

Reactions

Adenosine triphosphate + Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-phenylalanyl-tRNA(Phe)	details
Adenosine triphosphate + Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-Phenylalanyl-tRNA(Phe)	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Kodama K, Fukuzawa S, Sakamoto K, Nakayama H, Kigawa T, Yabuki T, Matsuda N, Shirouzu M, Takio K, Tachibana K, Yokoyama S: A new protein engineering approach combining chemistry and biology, part I; site-specific incorporation of 4-iodo-L-phenylalanine in vitro by using misacylated suppressor tRNAPhe. Chembiochem. 2006 Oct;7(10):1577-81. [PubMed:16969782 ]
Kotik-Kogan O, Moor N, Tworowski D, Safro M: Structural basis for discrimination of L-phenylalanine from L-tyrosine by phenylalanyl-tRNA synthetase. Structure. 2005 Dec;13(12):1799-807. [PubMed:16338408 ]

Enzyme Details

4. Tyrosine aminotransferase

General function:: Involved in 1-aminocyclopropane-1-carboxylate synthase activity
Specific function:: Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity towards phenylalanine.
Gene Name:: TAT
Uniprot ID:: P17735
Molecular weight:: 50398.895

Reactions

Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + Glutamic acid	details

References

Luong TN, Kirsch JF: A continuous coupled spectrophotometric assay for tyrosine aminotransferase activity with aromatic and other nonpolar amino acids. Anal Biochem. 1997 Nov 1;253(1):46-9. [PubMed:9356140 ]
Rege AA: Purification and characterization of a tyrosine aminotransferase from Crithidia fasciculata. Mol Biochem Parasitol. 1987 Aug;25(1):1-9. [PubMed:2890101 ]
De-Eknamkul W, Ellis BE: Behavior of Free Aromatic Amino Acid Pools in Rosmarinic Acid-Producing Cell Cultures of Anchusa officinalis L. Plant Physiol. 1989 Feb;89(2):429-33. [PubMed:16666560 ]
Patrizio M, Colucci M, Levi G: Human immunodeficiency virus type 1 Tat protein decreases cyclic AMP synthesis in rat microglia cultures. J Neurochem. 2001 Apr;77(2):399-407. [PubMed:11299302 ]

Enzyme Details

5. Aromatic-L-amino-acid decarboxylase

General function:: Involved in carboxy-lyase activity
Specific function:: Catalyzes the decarboxylation of L-3,4-dihydroxyphenylalanine (DOPA) to dopamine, L-5-hydroxytryptophan to serotonin and L-tryptophan to tryptamine.
Gene Name:: DDC
Uniprot ID:: P20711
Molecular weight:: 53893.755

Reactions

Phenylalanine → Phenylethylamine + Carbon dioxide	details

Enzyme Details

6. Lactoperoxidase

General function:: Involved in peroxidase activity
Specific function:: May contribute to airway host defense against infection.
Gene Name:: LPO
Uniprot ID:: P22079
Molecular weight:: 70983.6

Enzyme Details

7. Phenylalanine-4-hydroxylase

General function:: Involved in amino acid binding
Specific function:: Not Available
Gene Name:: PAH
Uniprot ID:: P00439
Molecular weight:: 51861.565

Reactions

Phenylalanine + Tetrahydrobiopterin + Oxygen → L-Tyrosine + 4a-Hydroxytetrahydrobiopterin	details
Tetrahydrobiopterin + Phenylalanine + Oxygen → 4a-Carbinolamine tetrahydrobiopterin + L-Tyrosine + Water	details

References

Thorolfsson M, Ibarra-Molero B, Fojan P, Petersen SB, Sanchez-Ruiz JM, Martinez A: L-phenylalanine binding and domain organization in human phenylalanine hydroxylase: a differential scanning calorimetry study. Biochemistry. 2002 Jun 18;41(24):7573-85. [PubMed:12056888 ]
Pueschel SM, Boylan J, Ellenbogen R: Studies on experimentally induced hyperphenylalaninemia. J Ment Defic Res. 1988 Aug;32 ( Pt 4):309-19. [PubMed:2975336 ]
Nagasaki Y, Matsubara Y, Takano H, Fujii K, Senoo M, Akanuma J, Takahashi K, Kure S, Hara M, Kanegae Y, Saito I, Narisawa K: Reversal of hypopigmentation in phenylketonuria mice by adenovirus-mediated gene transfer. Pediatr Res. 1999 Apr;45(4 Pt 1):465-73. [PubMed:10203136 ]
Fusetti F, Erlandsen H, Flatmark T, Stevens RC: Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria. J Biol Chem. 1998 Jul 3;273(27):16962-7. [PubMed:9642259 ]
Stokka AJ, Flatmark T: Substrate-induced conformational transition in human phenylalanine hydroxylase as studied by surface plasmon resonance analyses: the effect of terminal deletions, substrate analogues and phosphorylation. Biochem J. 2003 Feb 1;369(Pt 3):509-18. [PubMed:12379147 ]

Enzyme Details

8. Peroxiredoxin-6

General function:: Involved in antioxidant activity
Specific function:: Involved in redox regulation of the cell. Can reduce H(2)O(2) and short chain organic, fatty acid, and phospholipid hydroperoxides. May play a role in the regulation of phospholipid turnover as well as in protection against oxidative injury.
Gene Name:: PRDX6
Uniprot ID:: P30041
Molecular weight:: 25034.715

Reactions

Phenylalanine + Oxygen → 2-Phenylacetamide + Carbon dioxide	details

Enzyme Details

9. Myeloperoxidase

General function:: Involved in peroxidase activity
Specific function:: Part of the host defense system of polymorphonuclear leukocytes. It is responsible for microbicidal activity against a wide range of organisms. In the stimulated PMN, MPO catalyzes the production of hypohalous acids, primarily hypochlorous acid in physiologic situations, and other toxic intermediates that greatly enhance PMN microbicidal activity.
Gene Name:: MPO
Uniprot ID:: P05164
Molecular weight:: 83867.71

Enzyme Details

10. Aspartate aminotransferase, cytoplasmic

General function:: Involved in transferase activity, transferring nitrogenous groups
Specific function:: Plays a key role in amino acid metabolism (By similarity).
Gene Name:: GOT1
Uniprot ID:: P17174
Molecular weight:: 46247.14

Reactions

Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + Glutamic acid	details

Transporters

Enzyme Details

1. Large neutral amino acids transporter small subunit 2

General function:: Involved in transport
Specific function:: Sodium-independent, high-affinity transport of small and large neutral amino acids such as alanine, serine, threonine, cysteine, phenylalanine, tyrosine, leucine, arginine and tryptophan, when associated with SLC3A2/4F2hc. Acts as an amino acid exchanger. Has higher affinity for L-phenylalanine than LAT1 but lower affinity for glutamine and serine. L-alanine is transported at physiological concentrations. Plays a role in basolateral (re)absorption of neutral amino acids. Involved in the uptake of methylmercury (MeHg) when administered as the L-cysteine or D,L-homocysteine complexes, and hence plays a role in metal ion homeostasis and toxicity. Involved in the cellular activity of small molecular weight nitrosothiols, via the stereoselective transport of L-nitrosocysteine (L-CNSO) across the transmembrane. Plays an essential role in the reabsorption of neutral amino acids from the epithelial cells to the bloodstream in the kidney
Gene Name:: SLC7A8
Uniprot ID:: Q9UHI5
Molecular weight:: 58381.1

References

Shennan DB, Calvert DT, Travers MT, Kudo Y, Boyd CA: A study of L-leucine, L-phenylalanine and L-alanine transport in the perfused rat mammary gland: possible involvement of LAT1 and LAT2. Biochim Biophys Acta. 2002 Aug 19;1564(1):133-9. [PubMed:12101005 ]
Rossier G, Meier C, Bauch C, Summa V, Sordat B, Verrey F, Kuhn LC: LAT2, a new basolateral 4F2hc/CD98-associated amino acid transporter of kidney and intestine. J Biol Chem. 1999 Dec 3;274(49):34948-54. [PubMed:10574970 ]
Babu E, Kanai Y, Chairoungdua A, Kim DK, Iribe Y, Tangtrongsup S, Jutabha P, Li Y, Ahmed N, Sakamoto S, Anzai N, Nagamori S, Endou H: Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters. J Biol Chem. 2003 Oct 31;278(44):43838-45. Epub 2003 Aug 20. [PubMed:12930836 ]
Satoh S, Kimura T, Toda M, Maekawa M, Ono S, Narita H, Miyazaki H, Murayama T, Nomura Y: Involvement of L-type-like amino acid transporters in S-nitrosocysteine-stimulated noradrenaline release in the rat hippocampus. J Neurochem. 1997 Nov;69(5):2197-205. [PubMed:9349567 ]
Nemoto T, Shimma N, Horie S, Saito T, Okuma Y, Nomura Y, Murayama T: Involvement of the system L amino acid transporter on uptake of S-nitroso-L-cysteine, an endogenous S-nitrosothiol, in PC12 cells. Eur J Pharmacol. 2003 Jan 1;458(1-2):17-24. [PubMed:12498902 ]

Enzyme Details

2. Monocarboxylate transporter 10

General function:: Involved in transmembrane transport
Specific function:: Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:: SLC16A10
Uniprot ID:: Q8TF71
Molecular weight:: 55492.1

References

Kim DK, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H: The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location. Genomics. 2002 Jan;79(1):95-103. [PubMed:11827462 ]
Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508 ]

Only showing the first 10 proteins. There are 19 proteins in total.

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Showing metabocard for Phenylalanine (HMDB0000159)

MOL for HMDB0000159 (Phenylalanine)

3D MOL for HMDB0000159 (Phenylalanine)

3D SDF for HMDB0000159 (Phenylalanine)

3D-SDF for HMDB0000159 (Phenylalanine)

PDB for HMDB0000159 (Phenylalanine)

3D PDB for HMDB0000159 (Phenylalanine)

SMILES for HMDB0000159 (Phenylalanine)

INCHI for HMDB0000159 (Phenylalanine)

Structure for HMDB0000159 (Phenylalanine)

3D Structure for HMDB0000159 (Phenylalanine)

Experimental Collision Cross Sections

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

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GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes

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