VCP Associated Inclusion Body Myopathy and Paget Disease of Bone Knock-In Mouse Model Exhibits Tissue Pathology Typical of Human Disease

Dominant mutations in the valosin containing protein (VCP) gene cause inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD). We have generated a knock-in mouse model with the common R155H mutation. Mice demonstrate progressive muscle weakness start...

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Bibliografiske detaljer
Main Authors: Badadani, Mallikarjun, Nalbandian, Angèle, Watts, Giles D., Vesa, Jouni, Kitazawa, Masashi, Su, Hailing, Tanaja, Jasmin, Dec, Eric, Wallace, Douglas C., Mukherjee, Jogeshwar, Caiozzo, Vincent, Warman, Matthew, Kimonis, Virginia E.
Format: Artigo
Sprog:Inglês
Udgivet: Public Library of Science 2010
Fag:
Research Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2950155/
https://ncbi.nlm.nih.gov/pubmed/20957154
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0013183
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