Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice. (Q50471241)

14 March 2018

title

Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice. (English)

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14 March 2018

main subject

protein ubiquitination

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Conrad C Weihl

1

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14 March 2018

Sara E Miller

series ordinal

2

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14 March 2018

Phyllis I Hanson

series ordinal

3

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14 March 2018

Alan Pestronk

series ordinal

4

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14 March 2018

publication date

28 February 2007

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14 March 2018

published in

Human Molecular Genetics

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14 March 2018

volume

16

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14 March 2018

issue

8

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14 March 2018

page(s)

919-928

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14 March 2018

Molecular pathology and pathogenesis of inclusion-body myositis

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1 reference

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Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

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Autosomal dominant inclusion body myopathy, Paget disease of bone, and frontotemporal dementia

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Pathological consequences of VCP mutations on human striated muscle.

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Clinical delineation and localization to chromosome 9p13.3-p12 of a unique dominant disorder in four families: hereditary inclusion body myopathy, Paget disease of bone, and frontotemporal dementia

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Mutant valosin-containing protein causes a novel type of frontotemporal dementia

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21 January 2018

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Valosin-containing protein is a multi-ubiquitin chain-targeting factor required in ubiquitin-proteasome degradation

21 January 2018

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Interaction of U-box-type ubiquitin-protein ligases (E3s) with molecular chaperones

21 January 2018

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AAA ATPase p97/valosin-containing protein interacts with gp78, a ubiquitin ligase for endoplasmic reticulum-associated degradation

21 January 2018

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21 January 2018

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Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation

21 January 2018

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E-box sites and a proximal regulatory region of the muscle creatine kinase gene differentially regulate expression in diverse skeletal muscles and cardiac muscle of transgenic mice

21 January 2018

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Inclusion body myositis-like phenotype induced by transgenic overexpression of beta APP in skeletal muscle

21 January 2018

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21 January 2018

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Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin α2-deficient mice

21 January 2018

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Visualization of dystrophic muscle fibers in mdx mouse by vital staining with Evans blue: evidence of apoptosis in dystrophin-deficient muscle.

21 January 2018

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Animal models for muscular dystrophy show different patterns of sarcolemmal disruption.

21 January 2018

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21 January 2018

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21 January 2018

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Limb-girdle muscular dystrophy type 2H associated with mutation in TRIM32, a putative E3-ubiquitin-ligase gene

21 January 2018

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Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP

21 January 2018

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Caveolinopathies: mutations in caveolin-3 cause four distinct autosomal dominant muscle diseases

21 January 2018

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Limb-girdle muscular dystrophy (LGMD-1C) mutants of caveolin-3 undergo ubiquitination and proteasomal degradation. Treatment with proteasomal inhibitors blocks the dominant negative effect of LGMD-1C mutanta and rescues wild-type caveolin-3.

21 January 2018

1 reference

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Enhanced detection of congo-red-positive amyloid deposits in muscle fibers of inclusion body myositis and brain of Alzheimer's disease using fluorescence technique

21 January 2018

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Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice

21 January 2018

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDM037

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14 March 2018

PubMed ID

17329348

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14 March 2018