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Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia.

In the search for the mechanism by which hyperammonemia complicates propionic and methylmalonic acidemia the effects of a series of acyl-coenzyme A (CoA) derivatives were studied on the activity of N-acetylglutamate synthetase in rat liver mitochondria using acetyl-CoA as substrate. Propionyl-CoA wa...

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Detalhes bibliográficos
Publicado no:J Clin Invest
Principais autores: Coude, F X, Sweetman, L, Nyhan, W L
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 1979
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC371306/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/500823/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI109614
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