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Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants

Missense mutations of valosin-containing protein (VCP) cause an autosomal dominant disease known as inclusion body myopathy, Paget disease with frontotemporal dementia (IBMPFD) and other neurodegenerative disorders. The pathological mechanism of IBMPFD is not clear and there is no treatment. We show...

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Detalles Bibliográficos
Autores principales: Zhang, Ting, Mishra, Prashant, Hay, Bruce A, Chan, David, Guo, Ming
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5360448/
https://www.ncbi.nlm.nih.gov/pubmed/28322724
http://dx.doi.org/10.7554/eLife.17834