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The glycine N-acyltransferases, GLYAT and GLYATL1, contribute to the detoxification of isovaleryl-CoA - an in-silico and in vitro validation

Isovaleric acidemia (IVA), due to isovaleryl-CoA dehydrogenase (IVD) deficiency, results in the accumulation of isovaleryl-CoA, isovaleric acid and secondary metabolites. The increase in these metabolites decreases mitochondrial energy production and increases oxidative stress. This contributes to t...

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Hoofdauteurs: Stefan Kühn, Monray E. Williams, Marli Dercksen, Jörn Oliver Sass, Rencia van der Sluis
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: American Association for the Advancement of Science (AAAS) 2023-01-01
Reeks:Computational and Structural Biotechnology Journal
Onderwerpen:
Isovaleric acidemia
Glycine N-acyltransferase
Glycine conjugation
N-isovalerylglycine
Online toegang:http://www.sciencedirect.com/science/article/pii/S2001037023000430
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