FComEx: 2-Methylbutyrylglycine (PC000978)

Record Information

Version

1.0

Creation date

2015-10-09 22:33:40 UTC

Update date

2017-01-19 02:36:43 UTC

FoodComEx ID

PC000978

FoodDB Record

FDB021963

Chemical Information

Name

2-Methylbutyrylglycine

Description

2-Methylbutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine. The isolated excretion of 2-methylbutyrylglycine (2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (PubMedID 15615815). A number of previously unrecognized abnormal metabolites have been identified and quantitated in the urine of patients with propionyl-CoA carboxylase deficiency. These include the isoleucine metabolites, 2-methyl-3-hydroxybutyric acid and 2-methylacetoacetic acid. Additionally, 3-hydroxyvaleric acid and 3-oxovaleric acid were found, which may be products of the condensation of propionyl-CoA with acetyl-CoA catalyzed by 3-oxoacyl-CoA thiolases. Following a load of isoleucine, 2-methylbutyrylglycine was identified. This metabolite has not previously been observed in man. (PMID: 630060) [HMDB]

CAS Number

52320-67-9

Structure

MOL SDF PDB SMILES InChI

Synonyms

Synonym	Source
(2-methyl-butyrylamino)-acetate	hmdb
(2-methyl-butyrylamino)-acetic acid	hmdb
2-MBG	HMDB
2-Methylbutyryl glycine	HMDB
2-Methylbutyrylglycine	hmdb
a-Methylbutyrylglycine	hmdb
alpha-Methylbutyrylglycine	hmdb
N-(2-Methylbutyryl)glycine	hmdb
N-sec-Valerylglycine	hmdb

Chemical Formula

C7H13NO3

IUPAC name

2-(2-methylbutanamido)acetic acid

InChI Identifier

InChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10)

InChI Key

HOACIBQKYRHBOW-UHFFFAOYSA-N

Isomeric SMILES

CCC(C)C(=O)NCC(O)=O

Average Molecular Weight

159.183

Monoisotopic Molecular Weight

159.089543287

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as n-acyl-alpha amino acids. N-acyl-alpha amino acids are compounds containing an alpha amino acid which bears an acyl group at its terminal nitrogen atom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

N-acyl-alpha amino acids

Alternative Parents