Formation of 3‐hydroxyglutaric acid in glutaric aciduria type I: in vitro participation of medium chain acyl‐CoA dehydrogenase

Abstract 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‐methylg...

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Bibliographic Details

Main Authors:	Verena Peters, Marina Morath, Matthias Mack, Michael Liesert, Wolfgang Buckel, Georg F. Hoffmann, Jerry Vockley, Sandro Ghisla, Johannes Zschocke
Format:	Artigo
Language:	Inglês
Published:	Wiley 2019-05-01
Series:	JIMD Reports
Subjects:	3‐hydroxyglutaric acid acyl‐CoA dehydrogenase glutaconyl‐CoA glutaric aciduria type I glutaryl‐CoA
Online Access:	https://doi.org/10.1002/jmd2.12026
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