The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis

H Meyer, CC Weihl - Journal of cell science, 2014 - journals.biologists.com
The ATPase valosin-containing protein (VCP)/p97 has emerged as a central and important
element of the ubiquitin system. Together with a network of cofactors, it regulates an ever …

Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system

H Meyer, M Bug, S Bremer - Nature cell biology, 2012 - nature.com
The ATP-driven chaperone valosin-containing protein (VCP)/p97 governs critical steps in
ubiquitin-dependent protein quality control and intracellular signalling pathways. It …

Chaperone-assisted selective autophagy is essential for muscle maintenance

V Arndt, N Dick, R Tawo, M Dreiseidler, D Wenzel… - Current Biology, 2010 - cell.com
How are biological structures maintained in a cellular environment that constantly threatens
protein integrity? Here we elucidate proteostasis mechanisms affecting the Z disk, a protein …

During muscle atrophy, thick, but not thin, filament components are degraded by MuRF1-dependent ubiquitylation

S Cohen, JJ Brault, SP Gygi, DJ Glass… - Journal of Cell …, 2009 - rupress.org
Loss of myofibrillar proteins is a hallmark of atrophying muscle. Expression of muscle RING-
finger 1 (MuRF1), a ubiquitin ligase, is markedly induced during atrophy, and MuRF1 …

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

JS Ju, RA Fuentealba, SE Miller, E Jackson… - Journal of Cell …, 2009 - rupress.org
Mutations in valosin-containing protein (VCP) cause inclusion body myopathy (IBM), Paget's
disease of the bone, and frontotemporal dementia (IBMPFD). Patient muscle has …

Reversible inhibitor of p97, DBeQ, impairs both ubiquitin-dependent and autophagic protein clearance pathways

TF Chou, SJ Brown, D Minond… - Proceedings of the …, 2011 - National Acad Sciences
A specific small-molecule inhibitor of p97 would provide an important tool to investigate
diverse functions of this essential ATPase associated with diverse cellular activities (AAA) …

[HTML][HTML] VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations

NC Kim, E Tresse, RM Kolaitis, A Molliex, RE Thomas… - Neuron, 2013 - cell.com
Mutations in VCP cause multisystem degeneration impacting the nervous system, muscle,
and/or bone. Patients may present with ALS, Parkinsonism, frontotemporal dementia …

Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations

D Ritz, M Vuk, P Kirchner, M Bug, S Schütz, A Hayer… - Nature cell …, 2011 - nature.com
Abstract The AAA-ATPase VCP (also known as p97) cooperates with distinct cofactors to
process ubiquitylated proteins in different cellular pathways,,. VCP missense mutations …

The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks

K Acs, MS Luijsterburg, L Ackermann… - Nature structural & …, 2011 - nature.com
The accumulation of the human tumor suppressor 53BP1 at DNA damage sites requires the
ubiquitin ligases RNF8 and RNF168. As 53BP1 recognizes dimethylated Lys20 in histone …

UBXD7 binds multiple ubiquitin ligases and implicates p97 in HIF1α turnover

G Alexandru, J Graumann, GT Smith, NJ Kolawa… - Cell, 2008 - cell.com
Summary p97 is an ATP-dependent chaperone that plays an important role in endoplasmic
reticulum-associated degradation but whose connections to turnover of soluble proteins …