The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology.

The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology.

Valosin containing protein (VCP) mutations are the cause of hereditary inclusion body myopathy, Paget's disease of bone, frontotemporal dementia (IBMPFD). VCP gene mutations have also been linked to 2% of isolated familial amyotrophic lateral sclerosis (ALS). VCP is at the intersection of disru...

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Main Authors: Angèle Nalbandian, Katrina J Llewellyn, Masashi Kitazawa, Hong Z Yin, Mallikarjun Badadani, Negar Khanlou, Robert Edwards, Christopher Nguyen, Jogeshwar Mukherjee, Tahseen Mozaffar, Giles Watts, John Weiss, Virginia E Kimonis
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2012-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3460820?pdf=render

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