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Increased hepatic mitochondrial capacity in rats with hydroxy-cobalamin[c-lactam]-induced methylmalonic aciduria.

Treatment of rats with the vitamin B12 analogue hydroxy-cobalamin[c-lactam] (HCCL) impairs methylmalonyl-CoA mutase function and leads to methylmalonic aciduria due to intracellular accumulation of propionyl and methylmalonyl-CoA. Since accumulation of these acyl-CoAs disrupts normal cellular regula...

Ausführliche Beschreibung

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Bibliografische Detailangaben
Veröffentlicht in:J Clin Invest
Hauptverfasser: Krahenbuhl, S, Ray, D B, Stabler, S P, Allen, R H, Brass, E P
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society for Clinical Investigation 1990
Schlagworte:
Online-Zugang:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC329844/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/1701451/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI114942
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