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,sdg 3 - good health and well-being ,/dk/atira/pure/sustainabledevelopmentgoals/good_health_and_well_being
Faculty \ School:	Faculty of Medicine and Health Sciences > Norwich Medical School
UEA Research Groups:	Faculty of Medicine and Health Sciences > Research Groups > Musculoskeletal Medicine
Depositing User:	Pure Connector
Date Deposited:	06 Jan 2014 14:58
Last Modified:	24 Oct 2022 05:50
URI:	https://ueaeprints.uea.ac.uk/id/eprint/46864
DOI:	10.1002/mus.23522

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