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...

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Bibliographic Details
Main Authors: Krahenbuhl, S, Ray, D B, Stabler, S P, Allen, R H, Brass, E P
Format: Artigo
Language:Inglês
Published: 1990
Subjects:
Research Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC329844/
https://ncbi.nlm.nih.gov/pubmed/1701451
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