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

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...

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Detaylı Bibliyografya
Yayımlandı:JIMD Rep
Asıl Yazarlar: Peters, Verena, Morath, Marina, Mack, Matthias, Liesert, Michael, Buckel, Wolfgang, Hoffmann, Georg F., Vockley, Jerry, Ghisla, Sandro, Zschocke, Johannes
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: John Wiley & Sons, Inc. 2019
Konular:
Research Reports
Online Erişim:https://ncbi.nlm.nih.gov/pmc/articles/PMC6498835/
https://ncbi.nlm.nih.gov/pubmed/31240164
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jmd2.12026
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