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

Angèle Nalbandian; Katrina J Llewellyn; Mallikarjun Badadani; Hong Z Yin; Christopher Nguyen; Veeral Katheria; Giles Watts; Jogeshwar Mukherjee; Jouni Vesa; Vincent Caiozzo; Tahseen Mozaffar; John H Weiss; Virginia E Kimonis

doi:10.1002/mus.23522

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

Angèle Nalbandian, Katrina J Llewellyn, Mallikarjun Badadani, Hong Z Yin, Christopher Nguyen, Veeral Katheria, Giles Watts, Jogeshwar Mukherjee, Jouni Vesa, Vincent Caiozzo, Tahseen Mozaffar, John H Weiss, Virginia E Kimonis

Research output: Contribution to journal › Article › peer-review

59 Citations (Scopus)

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.

Original language	English
Pages (from-to)	260-70
Number of pages	11
Journal	Muscle & Nerve
Volume	47
Issue number	2
DOIs	https://doi.org/10.1002/mus.23522
Publication status	Published - Feb 2013

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

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

Access to Document

10.1002/mus.23522

Cite this

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

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.",

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",

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T1 - A progressive translational mouse model of human valosin-containing protein disease

T2 - the VCP(R155H/+) mouse

AU - Nalbandian, Angèle

AU - Llewellyn, Katrina J

AU - Badadani, Mallikarjun

AU - Yin, Hong Z

AU - Nguyen, Christopher

AU - Katheria, Veeral

AU - Watts, Giles

AU - Mukherjee, Jogeshwar

AU - Vesa, Jouni

AU - Caiozzo, Vincent

AU - Mozaffar, Tahseen

AU - Weiss, John H

AU - Kimonis, Virginia E

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AB - 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.

KW - Adenosine Triphosphatases

KW - Animals

KW - Brain

KW - Cell Cycle Proteins

KW - Disease Models, Animal

KW - Disease Progression

KW - Frontotemporal Dementia

KW - Mice

KW - Mice, Transgenic

KW - Motor Neurons

KW - Myositis, Inclusion Body

KW - Osteitis Deformans

KW - Spinal Cord

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DO - 10.1002/mus.23522

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