FComEx: Isovalerylglycine (PC000798)

Record Information

Version

1.0

Creation date

2015-10-09 22:32:42 UTC

Update date

2017-01-19 02:36:36 UTC

FoodComEx ID

PC000798

FoodDB Record

FDB022177

Chemical Information

Name

Isovalerylglycine

Description

Isovalerylglycine 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 Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA). IVA (OMIM/ McKusick 243500) is an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase (EC 1.3.99.10) gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine (IVG). It is very important to caution for false positive results when screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results. (PMID: 17850781) [HMDB]. Isovalerylglycine is a biomarker for the consumption of cheese.

CAS Number

16284-60-9

Structure

MOL SDF PDB SMILES InChI

Synonyms

Synonym	Source
[(3-methylbutanoyl)amino]acetate	hmdb
[(3-methylbutanoyl)amino]acetic acid	hmdb
2-(3-methylbutanoylamino)ethanoate	hmdb
2-(3-methylbutanoylamino)ethanoic acid	hmdb
3-methylbutyrylglycine	hmdb
Isopentanoylglycine	ChEBI
Isovalerylglycine	hmdb
N-Isopentanoylglycine	ChEBI
N-Isovaleroylglycine	hmdb
N-isovaleryl-glycine	hmdb

Chemical Formula

C7H13NO3

IUPAC name

2-(3-methylbutanamido)acetic acid

InChI Identifier

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

InChI Key

ZRQXMKMBBMNNQC-UHFFFAOYSA-N

Isomeric SMILES

CC(C)CC(=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