Mutations in valosin-containing protein (VCP) decrease ADP/ATP translocation across the mitochondrial membrane and impair energy metabolism in human neurons

Mutations in the gene encoding valosin-containing protein (VCP) lead to multisystem proteinopathies including frontotemporal dementia. We have previously shown that patient-derived VCP mutant fibroblasts exhibit lower mitochondrial membrane potential, uncoupled respiration, and reduced ATP levels. T...

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Detalles Bibliográficos
Autores principales: Ludtmann, Marthe H. R., Arber, Charles, Bartolome, Fernando, de Vicente, Macarena, Preza, Elisavet, Carro, Eva, Houlden, Henry, Gandhi, Sonia, Wray, Selina, Abramov, Andrey Y.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2017
Materias:
Neurobiology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5448124/
https://www.ncbi.nlm.nih.gov/pubmed/28360103
http://dx.doi.org/10.1074/jbc.M116.762898

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