VCP/p97 inhibitor CB-5083 modulates muscle pathology in a mouse model of VCP inclusion body myopathy

BACKGROUND: Pathogenic gain of function variants in Valosin-containing protein (VCP) cause a unique disease characterized by inclusion body myopathy with early-onset Paget disease of bone and frontotemporal dementia (also known as Multisystem proteinopathy (MSP)). Previous studies in drosophila mode...

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Detalles Bibliográficos
Autores principales: Cheng, Cheng, Weiss, Lan, Leinonen, Henri, Shmara, Alyaa, Yin, Hong Z., Ton, Timothy, Do, Annie, Lee, Jonathan, Ta, Lac, Mohanty, Eshanee, Vargas, Jesse, Weiss, John, Palczewski, Krzysztof, Kimonis, Virginia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8742393/
https://www.ncbi.nlm.nih.gov/pubmed/34998409
http://dx.doi.org/10.1186/s12967-021-03186-6

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