A Progressive Translational Mouse Model of Human VCP Disease: The VCP (R155H/+) Mouse

INTRODUCTION: Mutations in the valosin containing protein (VCP) gene cause hereditary Inclusion Body Myopathy (hIBM) associated with Paget disease of bone (PDB), and frontotemporal dementia (FTD). More recently they have been linked to 2% of familial ALS cases. A knock-in mouse model offers the oppo...

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Main Authors: Nalbandian, Angèle, Llewellyn, Katrina J., Badadani, Mallikarjun, Yin, Hong Z., Nguyen, Christopher, Katheria, Veeral, Watts, Giles, Mukherjee, Jogeshwar, Vesa, Jouni, Caiozzo, Vincent, Mozaffar, Tahseen, Weiss, John H., Kimonis, Virginia E.
Format: Artigo
Language:Inglês
Published: 2012
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3556223/
https://ncbi.nlm.nih.gov/pubmed/23169451
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/mus.23522
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