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Tricarboxylic acid cycle enzyme activities in a mouse model of methylmalonic aciduria.

Methylmalonic acidemia (MMA) is a propionate pathway disorder caused by dysfunction of the mitochondrial enzyme methylmalonyl-CoA mutase (MMUT). MMUT catalyzes the conversion of methylmalonyl-CoA to succinyl-CoA, an anaplerotic reaction which feeds into the tricarboxylic acid (TCA) cycle. As part of...

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Detalhes bibliográficos
Publicado no:Mol Genet Metab
Main Authors: Wongkittichote, Parith, Cunningham, Gary, Summar, Marshall L., Pumbo, Elena, Forny, Patrick, Baumgartner, Matthias R., Chapman, Kimberly A.
Formato: Artigo
Idioma:Inglês
Publicado em: 2019
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6903684/
https://ncbi.nlm.nih.gov/pubmed/31648943
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2019.10.007
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