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Pathogenic p62/SQSTM1 mutations impair energy metabolism through limitation of mitochondrial substrates

Abnormal mitochondrial function has been found in patients with frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Mutations in the p62 gene (also known as SQSTM1) which encodes the p62 protein have been reported in both disorders supporting the idea of an ALS/FTD continuum. In t...

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Detalles Bibliográficos
Autores principales: Bartolome, Fernando, Esteras, Noemi, Martin-Requero, Angeles, Boutoleau-Bretonniere, Claire, Vercelletto, Martine, Gabelle, Audrey, Le Ber, Isabelle, Honda, Tadashi, Dinkova-Kostova, Albena T., Hardy, John, Carro, Eva, Abramov, Andrey Y.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5431917/
https://www.ncbi.nlm.nih.gov/pubmed/28490746
http://dx.doi.org/10.1038/s41598-017-01678-4