Relief of CoA sequestration and restoration of mitochondrial function in a mouse model of propionic acidemia

Propionic acidemia (PA, OMIM 606054) is a devastating inborn error of metabolism arising from mutations that reduce the activity of the mitochondrial enzyme propionyl‐CoA carboxylase (PCC). The defects in PCC reduce the concentrations of nonesterified coenzyme A (CoASH), thus compromising mitochondr...

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Detalles Bibliográficos
Autores principales: Subramanian, Chitra, Frank, Matthew W., Tangallapally, Rajendra, Yun, Mi‐Kyung, White, Stephen W., Lee, Richard E., Rock, Charles O., Jackowski, Suzanne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2022
Materias:
Original Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10092110/
https://www.ncbi.nlm.nih.gov/pubmed/36251252
http://dx.doi.org/10.1002/jimd.12570

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