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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Autores principales: 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.
Formato: Artigo
Lenguaje:Inglês
Publicado: 2012
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Article
Acceso en línea: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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