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Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease
Mutations in valosin-containing protein (VCP) cause inclusion body myopathy (IBM), Paget's disease of the bone, and frontotemporal dementia (IBMPFD). Patient muscle has degenerating fibers, rimmed vacuoles (RVs), and sarcoplasmic inclusions containing ubiquitin and TDP-43 (TARDNA-binding protei...
Autores principales: | Ju, Jeong-Sun, Fuentealba, Rodrigo A., Miller, Sara E., Jackson, Erin, Piwnica-Worms, David, Baloh, Robert H., Weihl, Conrad C. |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
The Rockefeller University Press
2009
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2806317/ https://www.ncbi.nlm.nih.gov/pubmed/20008565 http://dx.doi.org/10.1083/jcb.200908115 |
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