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Showing metabocard for Isoleucine (HMDB0000172)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-07-07 20:53:57 UTC
HMDB IDHMDB0000172
Secondary Accession Numbers
  • HMDB00172
Metabolite Identification
Common NameIsoleucine
Description
Structure
Thumb
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000172 (Isoleucine)

  Mrv1652309272007352D          

  9  8  0  0  1  0            999 V2000
    5.2224   -0.0784    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    1.1592    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.7935   -0.9034    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    0.3341    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
    3.7935   -0.0784    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.3645   -0.0784    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    1.1592    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6500    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  9  1  0  0  0  0
  2  9  2  0  0  0  0
  5  3  1  1  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
  4  7  1  6  0  0  0
  5  9  1  0  0  0  0
  6  8  1  0  0  0  0
M  END
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3D MOL for HMDB0000172 (Isoleucine)

HMDB0000172
     RDKit          3D
L-Isoleucine
 22 21  0  0  0  0  0  0  0  0999 V2000
   -1.9483    1.3874   -0.2015 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7094   -0.0928   -0.0954 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2927   -0.4903   -0.3933 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.2345   -2.0164   -0.2386 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7018    0.0749    0.5808 C   0  0  2  0  0  0  0  0  0  0  0  0
    0.7185    1.5085    0.6256 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.0588   -0.3788    0.1206 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3447   -1.5974   -0.0157 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.0704    0.5308   -0.1787 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9257    1.5414   -0.7238 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0637    1.7905    0.8190 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1964    1.9172   -0.8110 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9488   -0.4780    0.9220 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3631   -0.6450   -0.8022 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0263   -0.2743   -1.4269 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4097   -2.4817   -0.9859 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2615   -2.4565   -0.3917 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0471   -2.2815    0.8132 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5202   -0.3244    1.5957 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3611    1.8613   -0.1223 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.2068    1.7723    1.5347 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4789    1.1329    0.5295 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  1  0
  5  7  1  0
  7  8  2  0
  7  9  1  0
  1 10  1  0
  1 11  1  0
  1 12  1  0
  2 13  1  0
  2 14  1  0
  3 15  1  6
  4 16  1  0
  4 17  1  0
  4 18  1  0
  5 19  1  1
  6 20  1  0
  6 21  1  0
  9 22  1  0
M  END
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3D SDF for HMDB0000172 (Isoleucine)

  Mrv1652309272007352D          

  9  8  0  0  1  0            999 V2000
    5.2224   -0.0784    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    1.1592    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.7935   -0.9034    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    0.3341    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
    3.7935   -0.0784    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.3645   -0.0784    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0791    1.1592    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6500    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5080    0.3341    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  9  1  0  0  0  0
  2  9  2  0  0  0  0
  5  3  1  1  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
  4  7  1  6  0  0  0
  5  9  1  0  0  0  0
  6  8  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000172

> <DATABASE_NAME>
hmdb

> <SMILES>
CC[C@H](C)[C@H](N)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1

> <INCHI_KEY>
AGPKZVBTJJNPAG-WHFBIAKZSA-N

> <FORMULA>
C6H13NO2

> <MOLECULAR_WEIGHT>
131.1729

> <EXACT_MASS>
131.094628665

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
22

> <JCHEM_AVERAGE_POLARIZABILITY>
14.11454343235902

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2S,3S)-2-amino-3-methylpentanoic acid

> <ALOGPS_LOGP>
-1.73

> <JCHEM_LOGP>
-1.5084066056000942

> <ALOGPS_LOGS>
-0.06

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
2.7913082723326537

> <JCHEM_PKA_STRONGEST_BASIC>
9.591345628135606

> <JCHEM_POLAR_SURFACE_AREA>
63.31999999999999

> <JCHEM_REFRACTIVITY>
34.093900000000005

> <JCHEM_ROTATABLE_BOND_COUNT>
3

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.14e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
L-isoleucine

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0000172 (Isoleucine)

HMDB0000172
     RDKit          3D
L-Isoleucine
 22 21  0  0  0  0  0  0  0  0999 V2000
   -1.9483    1.3874   -0.2015 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7094   -0.0928   -0.0954 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2927   -0.4903   -0.3933 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.2345   -2.0164   -0.2386 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7018    0.0749    0.5808 C   0  0  2  0  0  0  0  0  0  0  0  0
    0.7185    1.5085    0.6256 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.0588   -0.3788    0.1206 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3447   -1.5974   -0.0157 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.0704    0.5308   -0.1787 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9257    1.5414   -0.7238 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0637    1.7905    0.8190 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1964    1.9172   -0.8110 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9488   -0.4780    0.9220 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3631   -0.6450   -0.8022 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0263   -0.2743   -1.4269 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4097   -2.4817   -0.9859 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2615   -2.4565   -0.3917 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0471   -2.2815    0.8132 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5202   -0.3244    1.5957 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3611    1.8613   -0.1223 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.2068    1.7723    1.5347 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4789    1.1329    0.5295 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  1  0
  5  7  1  0
  7  8  2  0
  7  9  1  0
  1 10  1  0
  1 11  1  0
  1 12  1  0
  2 13  1  0
  2 14  1  0
  3 15  1  6
  4 16  1  0
  4 17  1  0
  4 18  1  0
  5 19  1  1
  6 20  1  0
  6 21  1  0
  9 22  1  0
M  END
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PDB for HMDB0000172 (Isoleucine)

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  O   UNK     0       9.748  -0.146   0.000  0.00  0.00           O+0
HETATM    2  O   UNK     0       8.415   2.164   0.000  0.00  0.00           O+0
HETATM    3  N   UNK     0       7.081  -1.686   0.000  0.00  0.00           N+0
HETATM    4  C   UNK     0       5.748   0.624   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0       7.081  -0.146   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0       4.414  -0.146   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0       5.748   2.164   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0       3.080   0.624   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0       8.415   0.624   0.000  0.00  0.00           C+0
CONECT    1    9                                                            
CONECT    2    9                                                            
CONECT    3    5                                                            
CONECT    4    5    6    7                                                  
CONECT    5    3    4    9                                                  
CONECT    6    4    8                                                       
CONECT    7    4                                                            
CONECT    8    6                                                            
CONECT    9    1    2    5                                                  
MASTER        0    0    0    0    0    0    0    0    9    0   16    0
END
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3D PDB for HMDB0000172 (Isoleucine)

COMPND    HMDB0000172
HETATM    1  C1  UNL     1      -1.948   1.387  -0.202  1.00  0.00           C  
HETATM    2  C2  UNL     1      -1.709  -0.093  -0.095  1.00  0.00           C  
HETATM    3  C3  UNL     1      -0.293  -0.490  -0.393  1.00  0.00           C  
HETATM    4  C4  UNL     1      -0.235  -2.016  -0.239  1.00  0.00           C  
HETATM    5  C5  UNL     1       0.702   0.075   0.581  1.00  0.00           C  
HETATM    6  N1  UNL     1       0.719   1.509   0.626  1.00  0.00           N  
HETATM    7  C6  UNL     1       2.059  -0.379   0.121  1.00  0.00           C  
HETATM    8  O1  UNL     1       2.345  -1.597  -0.016  1.00  0.00           O  
HETATM    9  O2  UNL     1       3.070   0.531  -0.179  1.00  0.00           O  
HETATM   10  H1  UNL     1      -2.926   1.541  -0.724  1.00  0.00           H  
HETATM   11  H2  UNL     1      -2.064   1.790   0.819  1.00  0.00           H  
HETATM   12  H3  UNL     1      -1.196   1.917  -0.811  1.00  0.00           H  
HETATM   13  H4  UNL     1      -1.949  -0.478   0.922  1.00  0.00           H  
HETATM   14  H5  UNL     1      -2.363  -0.645  -0.802  1.00  0.00           H  
HETATM   15  H6  UNL     1       0.026  -0.274  -1.427  1.00  0.00           H  
HETATM   16  H7  UNL     1       0.410  -2.482  -0.986  1.00  0.00           H  
HETATM   17  H8  UNL     1      -1.262  -2.457  -0.392  1.00  0.00           H  
HETATM   18  H9  UNL     1       0.047  -2.281   0.813  1.00  0.00           H  
HETATM   19  H10 UNL     1       0.520  -0.324   1.596  1.00  0.00           H  
HETATM   20  H11 UNL     1       1.361   1.861  -0.122  1.00  0.00           H  
HETATM   21  H12 UNL     1       1.207   1.772   1.535  1.00  0.00           H  
HETATM   22  H13 UNL     1       3.479   1.133   0.530  1.00  0.00           H  
CONECT    1    2   10   11   12
CONECT    2    3   13   14
CONECT    3    4    5   15
CONECT    4   16   17   18
CONECT    5    6    7   19
CONECT    6   20   21
CONECT    7    8    8    9
CONECT    9   22
END
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SMILES for HMDB0000172 (Isoleucine)

CC[C@H](C)[C@H](N)C(O)=O
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INCHI for HMDB0000172 (Isoleucine)

InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
Chemical FormulaC6H13NO2
Average Molecular Weight131.1729
Monoisotopic Molecular Weight131.094628665
IUPAC Name(2S,3S)-2-amino-3-methylpentanoic acid
Traditional NameL-isoleucine
CAS Registry Number73-32-5
SMILES
CC[C@H](C)[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1
InChI KeyAGPKZVBTJJNPAG-WHFBIAKZSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as isoleucine and derivatives. Isoleucine and derivatives are compounds containing isoleucine or a derivative thereof resulting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentIsoleucine and derivatives
Alternative Parents
Substituents
  • Isoleucine or derivatives
  • Alpha-amino acid
  • L-alpha-amino acid
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxide
  • Organopnictogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Organoleptic effect
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Biological roleIndustrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point285.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility35 mg/mLHuman Metabolome Project
LogP-1.70HANSCH,C ET AL. (1995)
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Astarita_neg129.030932474
[M-H]-Baker132.6230932474
[M-H]-McLean129.930932474
[M-H]-MetCCS_test_neg126.730932474
[M+H]+Baker134.830932474
[M+H]+McLean133.030932474
[M+H]+Astarita_pos128.730932474
[M-H]-Not Available130.8http://allccs.zhulab.cn/database/detail?ID=AllCCS00000078
[M+H]+Not Available136.967http://allccs.zhulab.cn/database/detail?ID=AllCCS00000078
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
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
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
Epilepsy
  1. 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 ]
Alzheimer's disease
  1. 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 ]
  2. 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 ]
Heart failure
  1. Norrelund H, Wiggers H, Halbirk M, Frystyk J, Flyvbjerg A, Botker HE, Schmitz O, Jorgensen JO, Christiansen JS, Moller N: Abnormalities of whole body protein turnover, muscle metabolism and levels of metabolic hormones in patients with chronic heart failure. J Intern Med. 2006 Jul;260(1):11-21. [PubMed:16789974 ]
Maple syrup urine disease
  1. Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. [PubMed:6422161 ]
  2. Yunus ZM, Kamaludin DA, Mamat M, Choy YS, Ngu L: Clinical and biochemical profiles of maple syrup urine disease in malaysian children. JIMD Rep. 2012;5:99-107. doi: 10.1007/8904_2011_105. Epub 2011 Dec 11. [PubMed:23430924 ]
  3. Barschak AG, Marchesan C, Sitta A, Deon M, Giugliani R, Wajner M, Vargas CR: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem. 2008 Mar;41(4-5):317-24. Epub 2007 Dec 5. [PubMed:18088602 ]
  4. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
  5. Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.
Colorectal cancer
  1. Ritchie SA, Ahiahonu PW, Jayasinghe D, Heath D, Liu J, Lu Y, Jin W, Kavianpour A, Yamazaki Y, Khan AM, Hossain M, Su-Myat KK, Wood PL, Krenitsky K, Takemasa I, Miyake M, Sekimoto M, Monden M, Matsubara H, Nomura F, Goodenowe DB: Reduced levels of hydroxylated, polyunsaturated ultra long-chain fatty acids in the serum of colorectal cancer patients: implications for early screening and detection. BMC Med. 2010 Feb 15;8:13. doi: 10.1186/1741-7015-8-13. [PubMed:20156336 ]
  2. 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 ]
  3. Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
Early preeclampsia
  1. 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
  1. 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 ]
  2. 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 ]
  3. 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 ]
Saccharopinuria
  1. Simell O, Visakorpi JK, Donner M: Saccharopinuria. Arch Dis Child. 1972 Feb;47(251):52-5. [PubMed:5018656 ]
Dihydrolipoamide Dehydrogenase Deficiency
  1. Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Branched-chain Keto Acid Dehydrogenase Kinase Deficiency
  1. Novarino G, El-Fishawy P, Kayserili H, Meguid NA, Scott EM, Schroth J, Silhavy JL, Kara M, Khalil RO, Ben-Omran T, Ercan-Sencicek AG, Hashish AF, Sanders SJ, Gupta AR, Hashem HS, Matern D, Gabriel S, Sweetman L, Rahimi Y, Harris RA, State MW, Gleeson JG: Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy. Science. 2012 Oct 19;338(6105):394-7. doi: 10.1126/science.1224631. Epub 2012 Sep 6. [PubMed:22956686 ]
Lipoyltransferase 1 Deficiency
  1. Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
Leukemia
  1. 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
  1. 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 ]
  2. 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 ]
  3. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Crohn's disease
  1. Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ]
  2. 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 ]
  3. 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 ]
Ulcerative colitis
  1. Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
Irritable bowel syndrome
  1. 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 ]
  2. 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 ]
Autism
  1. 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 ]
Perillyl alcohol administration for cancer treatment
  1. 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
  1. 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 ]
  2. OuYang D, Xu J, Huang H, Chen Z: Metabolomic profiling of serum from human pancreatic cancer patients using 1H NMR spectroscopy and principal component analysis. Appl Biochem Biotechnol. 2011 Sep;165(1):148-54. doi: 10.1007/s12010-011-9240-0. Epub 2011 Apr 20. [PubMed:21505807 ]
  3. Zhang L, Jin H, Guo X, Yang Z, Zhao L, Tang S, Mo P, Wu K, Nie Y, Pan Y, Fan D: Distinguishing pancreatic cancer from chronic pancreatitis and healthy individuals by (1)H nuclear magnetic resonance-based metabonomic profiles. Clin Biochem. 2012 Sep;45(13-14):1064-9. doi: 10.1016/j.clinbiochem.2012.05.012. Epub 2012 May 19. [PubMed:22613268 ]
Periodontal disease
  1. 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
  1. 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
  1. 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
  1. 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 ]
Missing teeth
  1. 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
  1. 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 ]
Autosomal dominant polycystic kidney disease
  1. Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDDB00167
Phenol Explorer Compound IDNot Available
FooDB IDFDB012397
KNApSAcK IDC00001374
Chemspider ID6067
KEGG Compound IDC00407
BioCyc IDILE
BiGG ID34887
Wikipedia LinkIsoleucine
METLIN ID5193
PubChem Compound6306
PDB IDNot Available
ChEBI ID17191
Food Biomarker OntologyNot Available
VMH IDILE_L
MarkerDB IDMDB00000083
Good Scents IDNot Available
References
Synthesis Reference Marvel, C. S. L-Isoleucine. Organic Syntheses (1941), 21 60-4.
Material Safety Data Sheet (MSDS)Not Available
General References

Enzymes

Enzyme Details
1. Branched-chain-amino-acid aminotransferase, cytosolic
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular weight:
38644.77
Reactions
Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + Glutamic aciddetails
References
  1. Goto M, Miyahara I, Hayashi H, Kagamiyama H, Hirotsu K: Crystal structures of branched-chain amino acid aminotransferase complexed with glutamate and glutarate: true reaction intermediate and double substrate recognition of the enzyme. Biochemistry. 2003 Apr 8;42(13):3725-33. [PubMed:12667063 ]
  2. Chen CD, Huang TF, Lin CH, Guan HH, Hsieh YC, Lin YH, Huang YC, Liu MY, Chang WC, Chen CJ: Purification, crystallization and preliminary X-ray crystallographic analysis of branched-chain aminotransferase from Deinococcus radiodurans. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2007 Jun 1;63(Pt 6):492-4. Epub 2007 May 5. [PubMed:17554170 ]
  3. Beck HC: Branched-chain fatty acid biosynthesis in a branched-chain amino acid aminotransferase mutant of Staphylococcus carnosus. FEMS Microbiol Lett. 2005 Feb 1;243(1):37-44. [PubMed:15667998 ]
  4. Thage BV, Rattray FP, Laustsen MW, Ardo Y, Barkholt V, Houlberg U: Purification and characterization of a branched-chain amino acid aminotransferase from Lactobacillus paracasei subsp. paracasei CHCC 2115. J Appl Microbiol. 2004;96(3):593-602. [PubMed:14962140 ]
  5. Madsen SM, Beck HC, Ravn P, Vrang A, Hansen AM, Israelsen H: Cloning and inactivation of a branched-chain-amino-acid aminotransferase gene from Staphylococcus carnosus and characterization of the enzyme. Appl Environ Microbiol. 2002 Aug;68(8):4007-14. [PubMed:12147502 ]
Enzyme Details
2. Branched-chain-amino-acid aminotransferase, mitochondrial
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:
BCAT2
Uniprot ID:
O15382
Molecular weight:
33776.315
Reactions
Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + Glutamic aciddetails
References
  1. Berger BJ, English S, Chan G, Knodel MH: Methionine regeneration and aminotransferases in Bacillus subtilis, Bacillus cereus, and Bacillus anthracis. J Bacteriol. 2003 Apr;185(8):2418-31. [PubMed:12670965 ]
Enzyme Details
3. Isoleucine--tRNA ligase, cytoplasmic
General function:
Involved in nucleotide binding
Specific function:
Not Available
Gene Name:
IARS
Uniprot ID:
P41252
Molecular weight:
144496.915
Reactions
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-isoleucyl-tRNA(Ile)details
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-Isoleucyl-tRNA(Ile)details
References
  1. Crasto CF, Forrest AK, Karoli T, March DR, Mensah L, O'Hanlon PJ, Nairn MR, Oldham MD, Yue W, Banwell MG, Easton CJ: Synthesis and activity of analogues of the isoleucyl tRNA synthetase inhibitor SB-203207. Bioorg Med Chem. 2003 Jul 3;11(13):2687-94. [PubMed:12788342 ]
  2. Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. [PubMed:12783542 ]
  3. Mock ML, Michon T, van Hest JC, Tirrell DA: Stereoselective incorporation of an unsaturated isoleucine analogue into a protein expressed in E. coli. Chembiochem. 2006 Jan;7(1):83-7. [PubMed:16397872 ]
  4. Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. [PubMed:14672940 ]
  5. Pezo V, Metzgar D, Hendrickson TL, Waas WF, Hazebrouck S, Doring V, Marliere P, Schimmel P, De Crecy-Lagard V: Artificially ambiguous genetic code confers growth yield advantage. Proc Natl Acad Sci U S A. 2004 Jun 8;101(23):8593-7. Epub 2004 May 26. [PubMed:15163798 ]
Enzyme Details
4. Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
Gene Name:
ACADSB
Uniprot ID:
P45954
Molecular weight:
47485.035
References
  1. Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [PubMed:15615815 ]
  2. Madsen PP, Kibaek M, Roca X, Sachidanandam R, Krainer AR, Christensen E, Steiner RD, Gibson KM, Corydon TJ, Knudsen I, Wanders RJ, Ruiter JP, Gregersen N, Andresen BS: Short/branched-chain acyl-CoA dehydrogenase deficiency due to an IVS3+3A>G mutation that causes exon skipping. Hum Genet. 2006 Feb;118(6):680-90. Epub 2005 Nov 30. [PubMed:16317551 ]
  3. Korman SH: Inborn errors of isoleucine degradation: a review. Mol Genet Metab. 2006 Dec;89(4):289-99. Epub 2006 Sep 6. [PubMed:16950638 ]
Enzyme Details
5. L-amino-acid oxidase
General function:
Involved in oxidoreductase activity
Specific function:
Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
Gene Name:
IL4I1
Uniprot ID:
Q96RQ9
Molecular weight:
65327.26
Reactions
Isoleucine + Water + Oxygen → 3-Methyl-2-oxovaleric acid + Ammonia + Hydrogen peroxidedetails
Enzyme Details
6. Isoleucine--tRNA ligase, mitochondrial
General function:
Involved in nucleotide binding
Specific function:
Not Available
Gene Name:
IARS2
Uniprot ID:
Q9NSE4
Molecular weight:
113790.565
Reactions
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-isoleucyl-tRNA(Ile)details
Adenosine triphosphate + Isoleucine + tRNA(Ile) → Adenosine monophosphate + Pyrophosphate + L-Isoleucyl-tRNA(Ile)details
References
  1. Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. [PubMed:12783542 ]
  2. Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. [PubMed:14672940 ]
  3. Zhu B, Zhao MW, Eriani G, Wang ED: A present-day aminoacyl-tRNA synthetase with ancestral editing properties. RNA. 2007 Jan;13(1):15-21. Epub 2006 Nov 9. [PubMed:17095543 ]
  4. Fukunaga R, Yokoyama S: Crystal structure of leucyl-tRNA synthetase from the archaeon Pyrococcus horikoshii reveals a novel editing domain orientation. J Mol Biol. 2005 Feb 11;346(1):57-71. Epub 2004 Dec 19. [PubMed:15663927 ]
  5. Fukunaga R, Yokoyama S: Structural basis for substrate recognition by the editing domain of isoleucyl-tRNA synthetase. J Mol Biol. 2006 Jun 16;359(4):901-12. Epub 2006 Apr 25. [PubMed:16697013 ]

Transporters

Enzyme Details
1. 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
  1. 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 ]