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Impaired Protein Aggregate Handling and Clearance Underlie the Pathogenesis of p97/VCP-associated Disease

Mutations in p97/VCP cause the multisystem disease inclusion body myopathy, Paget disease of the bone and frontotemporal dementia (IBMPFD). p97/VCP is a member of the AAA+ (ATPase associated with a variety of activities) protein family and has been implicated in multiple cellular processes. One path...

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Detalhes bibliográficos
Main Authors: Ju, Jeong-Sun, Miller, Sara E., Hanson, Phyllis I., Weihl, Conrad C.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2008
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2573070/
https://ncbi.nlm.nih.gov/pubmed/18715868
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M805517200
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