A progressive translational mouse model of human valosin-containing protein disease:the VCP(R155H/+) mouse

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 and Kimonis, Virginia E (2013) A progressive translational mouse model of human valosin-containing protein disease:the VCP(R155H/+) mouse. Muscle & Nerve, 47 (2). pp. 260-70. ISSN 0148-639X

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Abstract

Mutations in the valosin-containing protein (VCP) gene cause hereditary inclusion body myopathy (IBM) associated with Paget disease of bone (PDB), and frontotemporal dementia (FTD). More recently, these mutations have been linked to 2% of familial amyotrophic lateral sclerosis (ALS) cases. A knock-in mouse model offers the opportunity to study VCP-associated pathogenesis.

Item Type: Article
Additional Information: Copyright © 2012 Wiley Periodicals, Inc.
Uncontrolled Keywords: adenosine triphosphatases,animals,brain,cell cycle proteins,disease models, animal,disease progression,frontotemporal dementia,mice,mice, transgenic,motor neurons,myositis, inclusion body,osteitis deformans,spinal cord
Faculty \ School: Faculty of Medicine and Health Sciences > Norwich Medical School
University of East Anglia > Faculty of Medicine and Health Sciences > Research Groups > Musculoskeletal Science
Depositing User: Pure Connector
Date Deposited: 06 Jan 2014 14:58
Last Modified: 25 Jul 2018 09:20
URI: https://ueaeprints.uea.ac.uk/id/eprint/46864
DOI: 10.1002/mus.23522

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