Structural basis for defective membrane targeting of mutant enzyme in human VLCAD deficiency

Very long-chain acyl-CoA dehydrogenase (VLCAD) is an inner mitochondrial membrane enzyme that catalyzes the first and rate-limiting step of long-chain fatty acid oxidation. Point mutations in human VLCAD can produce an inborn error of metabolism called VLCAD deficiency that can lead to severe pathop...

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Detalles Bibliográficos
Autores principales: Prew, Michelle S., Camara, Christina M., Botzanowski, Thomas, Moroco, Jamie A., Bloch, Noah B., Levy, Hannah R., Seo, Hyuk-Soo, Dhe-Paganon, Sirano, Bird, Gregory H., Herce, Henry D., Gygi, Micah A., Escudero, Silvia, Wales, Thomas E., Engen, John R., Walensky, Loren D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2022
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9237092/
https://www.ncbi.nlm.nih.gov/pubmed/35760926
http://dx.doi.org/10.1038/s41467-022-31466-2

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