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3-methylglutaconic acid

Known as: 3-methyl-2-pentenedioic acid 
National Institutes of Health

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Glutarates

Papers overview

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2010
2010
OPA3, mutated in 3-methylglutaconic aciduria type III, encodes two transcripts targeted primarily to mitochondria.
2004
2004
Direct nonisotopic assay of 3-methylglutaconyl-CoA hydratase in cultured human skin fibroblasts to specifically identify patients with 3-methylglutaconic aciduria type I.
3-Methylglutaconic aciduria (3MGA) type I (McKusick 250950) is biochemically characterized by increased excretion of 3… 
1999
1999
What is the origin of 3-methylglutaconic acid?
Increased urinary excretion of 3-methylglutaconic acid (3-MGC) and to a lesser extent 3-methylglutaric acid (3-MGR) is a not… 
1996
1996
Combined 3-methylglutaconic and 3-hydroxy-3-methylglutaric aciduria with endocardial fibroelastosis and dilatative cardiomyopathy in male and female siblings with partial deficiency of complex II/III…
We report on 2 children, brother and sister, who presented with cardiomyopathy and muscular hypotonia at the age of B months… 
1995
1995
3-Methylglutaconic Acidemia in Smith-Lemli-Opitz Syndrome
ABSTRACT: The branched-chain organic acid, 3-methylglutaconic acid, is an intermediate (as the CoA thioester) in the leucine… 
1995
1995
Molecular prenatal diagnosis of 3‐hydroxy−3‐methylglutaryl coa lyase deficiency
We report the first molecular prenatal diagnosis of 3‐hydroxy‐3‐methylglutaryl CoA lyase (HL) deficiency. The proband had a… 
1993
1993
3‐Methylglutaconic aciduria in “optic atrophy plus”
Behr's syndrome consists of recessively inherited infantile optic atrophy, together with chronic neurological disturbances such… 
1992
1992
Behr's syndrome and 3-methylglutaconic aciduria.
1992
1992
3-methylglutaconic aciduria in a patient with a disturbed mitochondrial energy metabolism
Sir: Three types of 3-methylglutaconic aciduria (McKusick 25095) can be distinguished [2]: 1. Patients with a massive excretion… 
1983
1983
3-Methylglutaconic aciduria in two infants.
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