Formation of 3-hydroxyglutaric acid in glutaric aciduria type I: in vitro participation of medium chain acyl-CoA dehydrogenase.
3‐hydroxyglutaric acid
acyl‐CoA dehydrogenase
glutaconyl‐CoA
glutaric aciduria type I
glutaryl‐CoA
Journal
JIMD reports
ISSN: 2192-8304
Titre abrégé: JIMD Rep
Pays: United States
ID NLM: 101568557
Informations de publication
Date de publication:
May 2019
May 2019
Historique:
received:
26
11
2018
accepted:
28
01
2019
entrez:
27
6
2019
pubmed:
27
6
2019
medline:
27
6
2019
Statut:
epublish
Résumé
3-Hydroxyglutaric acid (3-OH-GA) in urine has been identified as the most reliable diagnostic marker for glutaric aciduria type I (GA I). We showed that hydratation of glutaconyl-CoA to 3-hydroxyglutaryl-CoA, which is subsequently hydrolyzed to 3-OH-GA, is efficiently catalyzed by 3-methylglutaconyl-CoA hydratase (3-MGH). We have now investigated whether mitochondrial acyl-CoA-dehydrogenases can convert glutaryl-CoA to glutaconyl-CoA. Short-chain acyl-CoA dehydrogenase (SCAD), medium-chain acyl-CoA dehydrogenase (MCAD), and long-chain acyl-CoA dehydrogenase (LCAD) accepted glutaryl-CoA as a substrate. The highest
Identifiants
pubmed: 31240164
doi: 10.1002/jmd2.12026
pii: JMD212026
pmc: PMC6498835
doi:
Types de publication
Journal Article
Langues
eng
Pagination
30-34Subventions
Organisme : NIDDK NIH HHS
ID : R01 DK109907
Pays : United States
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