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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2006-05-22 14:17:39 UTC

Update Date

2023-02-21 17:16:13 UTC

HMDB ID

HMDB0002166

Secondary Accession Numbers

HMDB02166

Metabolite Identification

Common Name

(S)-beta-Aminoisobutyric acid

Description

beta-Aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine. The concentration of beta-aminoisobutyric acid is normally low in urine as beta-aminoisobutyric acid is further catabolized by beta-aminoisobutyrate aminotransferases to methylmalonic acid semialdehyde and propionyl-CoA. beta-Aminoisobutyric acid occurs in two isomeric forms and both enantiomers of beta-aminoisobutyric acid can be detected in human urine and plasma. In plasma, the S-enantiomer is the predominant type due to active renal reabsorption. In contrast, urine almost exclusively contains the R-enantiomer of beta-aminoisobutyric acid, which is eliminated both by filtration and tubular secretion. Persistently increased levels of beta-aminoisobutyric acid have been observed in individuals with a deficiency of R (-)-beta-aminoisobutyrate-pyruvate aminotransferase. In addition, transient high levels of beta-aminoisobutyric acid have been observed under a variety of pathological conditions such as lead poisoning, starvation, in total body irradiation, and in a number of malignancies. The S-enantiomer of beta-aminoisobutyric acid is predominantly derived from the catabolism of valine. It has been suggested that altered homeostasis of beta-alanine underlies some of the clinical abnormalities encountered in patients with a dihydropyrimidine dehydrogenase (DPD) deficiency. DPD constitutes the first step of the pyrimidine degradation pathway, in which the pyrimidine bases uracil and thymine are catabolized to beta-alanine and the R-enantiomer of beta-aminoisobutyric acid respectively. In normal individuals with an intact pyrimidine degradation pathway, R-methylmalonic acid semialdehyde can be synthesized directly from the catabolism of thymine. Hence, there might be less cross-over between the valine and thymine pathway, allowing the conversion of S-methylmalonic acid semialdehyde into S-beta-aminoisobutyric acid and the subsequent accumulation of S-beta-aminoisobutyric acid in plasma (PMID: 14705962 , 14292857 , 14453202 ).

Structure

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

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(S)-3-Amino-2-methylpropanoic acid	ChEBI
(S)-3-Amino-isobutanoic acid	ChEBI
(S)-3-Amino-isobutyric acid	ChEBI
L-3-Amino-isobutanoic acid	ChEBI
L-3-Amino-isobutyric acid	ChEBI
(S)-3-Aminoisobutyrate	Kegg
L-3-Aminoisobutyrate	Kegg
(S)-3-Aminoisobutanoate	Kegg
(S)-3-Amino-2-methylpropanoate	Kegg
(S)-beta-Aminoisobutyrate	Kegg
(S)-3-Amino-isobutanoate	Generator
(S)-3-Amino-isobutyrate	Generator
L-3-Amino-isobutanoate	Generator
L-3-Amino-isobutyrate	Generator
(S)-3-Aminoisobutyric acid	Generator
L-3-Aminoisobutyric acid	Generator
(S)-3-Aminoisobutanoic acid	Generator
(S)-b-Aminoisobutyrate	Generator
(S)-b-Aminoisobutyric acid	Generator
(S)-Β-aminoisobutyrate	Generator
(S)-Β-aminoisobutyric acid	Generator
(S)-beta-Aminoisobutyric acid	ChEBI
(+)-a-Methyl-b-alanine	HMDB
(+)-alpha-Methyl-beta-alanine	HMDB
(+)-b-Aminoisobutyric acid	HMDB
(+)-beta-Aminoisobutyric acid	HMDB
(S)-3-amino-2-Methyl-propanoate	HMDB
(S)-3-amino-2-Methyl-propanoic acid	HMDB
L-2-Methyl-b-alanine	HMDB
L-2-Methyl-beta-alanine	HMDB
L-3-amino-2-Methylpropanoate	HMDB
L-3-amino-2-Methylpropanoic acid	HMDB
L-3-amino-2-Methylpropionic acid	HMDB
L-b-Aminoisobutyrate	HMDB
L-b-Aminoisobutyric acid	HMDB
L-beta-Aminoisobutyrate	HMDB
L-beta-Aminoisobutyric acid	HMDB
S-b-Aminoisobutyrate	HMDB
S-beta-Aminoisobutyrate	HMDB
S-beta-Aminoisobutyric acid	HMDB

Chemical Formula

C₄H₉NO₂

Average Molecular Weight

103.1198

Monoisotopic Molecular Weight

103.063328537

IUPAC Name

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

Traditional Name

L-β-aminoisobutyric acid

CAS Registry Number

4249-19-8

SMILES

C[C@@H](CN)C(O)=O

InChI Identifier

InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1

InChI Key

QCHPKSFMDHPSNR-VKHMYHEASA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as beta amino acids and derivatives. These are amino acids having a (-NH2) group attached to the beta carbon atom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Beta amino acids and derivatives

Alternative Parents

Substituents

Beta amino acid or derivatives
Amino acid
Carboxylic acid
Monocarboxylic acid or derivatives
Amine
Organic oxide
Hydrocarbon derivative
Primary amine
Organooxygen compound
Organonitrogen compound
Organopnictogen compound
Primary aliphatic amine
Organic oxygen compound
Carbonyl group
Organic nitrogen compound
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

beta-amino acid (CHEBI:33094 )
3-aminoisobutyric acid (CHEBI:33094 )
Amino fatty acids (LMFA01100050 )

Ontology

Not Available

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	175 - 177 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	367 g/L	ALOGPS
logP	-3	ALOGPS
logP	-2.6	ChemAxon
logS	0.55	ALOGPS
pKa (Strongest Acidic)	4.17	ChemAxon
pKa (Strongest Basic)	10.32	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	2	ChemAxon
Refractivity	25.28 m³·mol⁻¹	ChemAxon
Polarizability	10.42 Å³	ChemAxon
Number of Rings	0	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]+	121.515	31661259
DarkChem	[M-H]-	116.429	31661259
DeepCCS	[M+H]+	120.309	30932474
DeepCCS	[M-H]-	117.41	30932474
DeepCCS	[M-2H]-	153.942	30932474
DeepCCS	[M+Na]+	128.806	30932474
AllCCS	[M+H]+	126.7	32859911
AllCCS	[M+H-H2O]+	122.5	32859911
AllCCS	[M+NH4]+	130.7	32859911
AllCCS	[M+Na]+	131.9	32859911
AllCCS	[M-H]-	124.7	32859911
AllCCS	[M+Na-2H]-	128.7	32859911
AllCCS	[M+HCOO]-	133.0	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
(S)-beta-Aminoisobutyric acid	C[C@@H](CN)C(O)=O	1840.6	Standard polar	33892256
(S)-beta-Aminoisobutyric acid	C[C@@H](CN)C(O)=O	1015.3	Standard non polar	33892256
(S)-beta-Aminoisobutyric acid	C[C@@H](CN)C(O)=O	1032.5	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
(S)-beta-Aminoisobutyric acid,1TMS,isomer #1	C[C@@H](CN)C(=O)O[Si](C)(C)C	1051.8	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,1TMS,isomer #2	C[C@@H](CN[Si](C)(C)C)C(=O)O	1206.4	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TMS,isomer #1	C[C@@H](CN[Si](C)(C)C)C(=O)O[Si](C)(C)C	1230.4	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TMS,isomer #1	C[C@@H](CN[Si](C)(C)C)C(=O)O[Si](C)(C)C	1249.7	Standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TMS,isomer #1	C[C@@H](CN[Si](C)(C)C)C(=O)O[Si](C)(C)C	1331.9	Standard polar	33892256
(S)-beta-Aminoisobutyric acid,2TMS,isomer #2	C[C@@H](CN([Si](C)(C)C)[Si](C)(C)C)C(=O)O	1412.2	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TMS,isomer #2	C[C@@H](CN([Si](C)(C)C)[Si](C)(C)C)C(=O)O	1357.2	Standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TMS,isomer #2	C[C@@H](CN([Si](C)(C)C)[Si](C)(C)C)C(=O)O	1608.9	Standard polar	33892256
(S)-beta-Aminoisobutyric acid,3TMS,isomer #1	C[C@@H](CN([Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C	1464.4	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,3TMS,isomer #1	C[C@@H](CN([Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C	1414.9	Standard non polar	33892256
(S)-beta-Aminoisobutyric acid,3TMS,isomer #1	C[C@@H](CN([Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C	1339.4	Standard polar	33892256
(S)-beta-Aminoisobutyric acid,1TBDMS,isomer #1	C[C@@H](CN)C(=O)O[Si](C)(C)C(C)(C)C	1282.3	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,1TBDMS,isomer #2	C[C@@H](CN[Si](C)(C)C(C)(C)C)C(=O)O	1450.9	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TBDMS,isomer #1	C[C@@H](CN[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1668.6	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TBDMS,isomer #1	C[C@@H](CN[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1679.3	Standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TBDMS,isomer #1	C[C@@H](CN[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1627.1	Standard polar	33892256
(S)-beta-Aminoisobutyric acid,2TBDMS,isomer #2	C[C@@H](CN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O	1828.1	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TBDMS,isomer #2	C[C@@H](CN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O	1761.4	Standard non polar	33892256
(S)-beta-Aminoisobutyric acid,2TBDMS,isomer #2	C[C@@H](CN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O	1785.6	Standard polar	33892256
(S)-beta-Aminoisobutyric acid,3TBDMS,isomer #1	C[C@@H](CN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	2095.5	Semi standard non polar	33892256
(S)-beta-Aminoisobutyric acid,3TBDMS,isomer #1	C[C@@H](CN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	2054.2	Standard non polar	33892256
(S)-beta-Aminoisobutyric acid,3TBDMS,isomer #1	C[C@@H](CN([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1764.4	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-beta-Aminoisobutyric acid GC-MS (Non-derivatized) - 70eV, Positive	splash10-001i-9000000000-59e52601f90d8e636d61	2017-09-01	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-beta-Aminoisobutyric acid GC-MS (1 TMS) - 70eV, Positive	splash10-00ar-9200000000-0eb92539979a9fa36dbd	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-beta-Aminoisobutyric acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 1V, negative-QTOF	splash10-0udi-1900000000-aaccebe5d4a868bc32bf	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 2V, negative-QTOF	splash10-0udi-2900000000-4f4e49fe169d5c21efc1	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 2V, negative-QTOF	splash10-0udi-4900000000-f32e82420f5efb60456e	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 3V, negative-QTOF	splash10-0uk9-6900000000-cb0ef82b397e24372142	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 3V, negative-QTOF	splash10-0uk9-8900000000-31fc31847c8771693d15	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 3V, negative-QTOF	splash10-0fk9-9600000000-6379643466e30660a05b	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 4V, negative-QTOF	splash10-00di-9300000000-97e8a7125780e41fceaf	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 5V, negative-QTOF	splash10-05fr-9200000000-8f0488fe62603d121536	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 5V, negative-QTOF	splash10-0ab9-9100000000-84e07fbee3e332502265	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 6V, negative-QTOF	splash10-0a4i-9000000000-3634b730902e2e080b56	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid n/a 7V, negative-QTOF	splash10-00di-9000000000-1b9078e3902a11473fa0	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 0V, positive-QTOF	splash10-0udi-1900000000-76d19f44560e6b1b6f80	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 0V, positive-QTOF	splash10-0udi-1900000000-bd4e85f35b448a2b7540	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 0V, positive-QTOF	splash10-0udi-2900000000-b1371ec53be10ec9c411	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 1V, positive-QTOF	splash10-0udi-3900000000-d16b3475fcdfed5038d5	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 1V, positive-QTOF	splash10-0udi-4900000000-cd34930bbcb805c7525f	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 1V, positive-QTOF	splash10-0udr-5900000000-4b73ac1046680185a981	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 1V, positive-QTOF	splash10-0udr-6900000000-a911da27982a560a6cf1	2020-07-22	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid Orbitrap 1V, positive-QTOF	splash10-0udr-8900000000-91a255aae27e4e4aabb8	2020-07-22	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid 10V, Positive-QTOF	splash10-0f79-9300000000-30669ed04aadf3d3f040	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid 20V, Positive-QTOF	splash10-000l-9000000000-ca65637476e64152c44e	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid 40V, Positive-QTOF	splash10-0006-9000000000-04d1648391d3f90db226	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid 10V, Negative-QTOF	splash10-0udi-3900000000-ec54fcce079024a62344	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid 20V, Negative-QTOF	splash10-0zfr-9500000000-0394f399cdf5df93c5df	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-beta-Aminoisobutyric acid 40V, Negative-QTOF	splash10-0a4l-9000000000-d0c495c908ed905558a2	2015-09-15	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
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

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
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

Not Available

Biospecimen Locations

Blood
Feces
Urine

Tissue Locations

Not Available

Pathways

Name	SMPDB/PathBank	KEGG
Valine, leucine and isoleucine degradation	Not Available
Beta-Ketothiolase Deficiency		Not Available
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency		Not Available
Propionic Acidemia		Not Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	1.03 +/- 0.34 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	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)	Both	Normal	24029555	details
Urine	Detected and Quantified	2.71-57.80 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	11.31 +/- 16.96 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	14.70 +/- 38.44 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	1.24-32.35 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	7.0 +/- 6.6 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	<34.048 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	10.0 +/- 9.0 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	4.3 +/- 2.9 umol/mmol creatinine	Children (1-13 years old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	2.04-354.79 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	<110.86 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	1.70-117.53 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details

Abnormal Concentrations

Not Available

Associated Disorders and Diseases

Disease References

None

Associated OMIM IDs

None

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022878

KNApSAcK ID

Not Available

Chemspider ID

388543

KEGG Compound ID

C03284

BioCyc ID

CPD-466

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

6520

PubChem Compound

439434

PDB ID

Not Available

ChEBI ID

33094

Food Biomarker Ontology

Not Available

VMH ID

3AIB

MarkerDB ID

Not Available

Good Scents ID

Not Available

References

Synthesis Reference

Alauddin, Mian M.; Fissekis, John D.; Conti, Peter S. a-Alkylation of amino acid derivatives: synthesis and chiral resolution of [11C]b-aminoisobutyric acid. Nuclear Medicine and Biology (1997), 24(8), 771-775.

Material Safety Data Sheet (MSDS)

Not Available

General References

Van Kuilenburg AB, Stroomer AE, Van Lenthe H, Abeling NG, Van Gennip AH: New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for beta-aminoisobutyric acid? Biochem J. 2004 Apr 1;379(Pt 1):119-24. [PubMed:14705962 ]
Roe CR, Struys E, Kok RM, Roe DS, Harris RA, Jakobs C: Methylmalonic semialdehyde dehydrogenase deficiency: psychomotor delay and methylmalonic aciduria without metabolic decompensation. Mol Genet Metab. 1998 Sep;65(1):35-43. [PubMed:9787093 ]
KAKIMOTO Y, KANAZAWA A, SANO I: IDENTIFICATION OF D(-)-BETA-AMINOISOBUTYRIC ACID IN HUMAN LIVER. Biochim Biophys Acta. 1965 Feb 15;97:376-7. [PubMed:14292857 ]
KAKIMOTO Y, ARMSTRONG MD: The preparation and isolation of D-(-)-beta-aminoisobutyric acid. J Biol Chem. 1961 Dec;236:3283-6. [PubMed:14453202 ]

Enzymes

Enzyme Details

1. 4-aminobutyrate aminotransferase, mitochondrial

General function:: Involved in 4-aminobutyrate transaminase activity
Specific function:: Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
Gene Name:: ABAT
Uniprot ID:: P80404
Molecular weight:: 56438.405

Reactions

(S)-beta-Aminoisobutyric acid + Oxoglutaric acid → 2-Methyl-3-oxopropanoic acid + Glutamic acid	details
(S)-beta-Aminoisobutyric acid + Oxoglutaric acid → (S)-Methylmalonic acid semialdehyde + Glutamic acid	details

Showing metabocard for (S)-beta-Aminoisobutyric acid (HMDB0002166)

MOL for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

3D MOL for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

3D SDF for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

3D-SDF for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

PDB for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

3D PDB for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

SMILES for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

INCHI for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

Structure for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

3D Structure for HMDB0002166 ((S)-beta-Aminoisobutyric acid)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes

Reactions