2-ethylhydracrylic acid

newly described urinary organic acid; a metabolite of L-isoleucine; structure

Also Known As:

2-(hydroxymethyl)butyric acid; 2-ethyl-3-hydroxypropionic acid; Butanoic acid, 2-(hydroxymethyl)-

Networked: 2 relevant articles (0 outcomes, 0 trials/studies)

1.	Maple Syrup Urine Disease 12/15/1976 - "2. Urinary excretion of 2-ethylhydracrylic acid is variably increased in defects of isoleucine oxidation at distal steps in the catabolic pathway (3-oxoacyl-CoA thiolase deficiency and methylmalonyl-CoA mutase deficiency) and is diminished when proximal steps of the oxidative pathway are blocked as in branched-chain oxo acid decarboxylase deficiency ('maple-syrup-urine' disease). "
2.	2-Methylbutyryl-CoA Dehydrogenase Deficiency 03/01/2005 - "Excretion of 2-ethylhydracrylic acid (2-EHA), an intermediate formed in the normally minor R-pathway of L-isoleucine oxidation, has not previously been described in SBCADD. "