Literature DB >> 25146396

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

Hemmo Meyer1, Conrad C Weihl2.   

Abstract

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-expanding range of processes that stretch into almost every aspect of cellular physiology. Its main role in proteostasis and key functions in signaling pathways are of relevance to degenerative diseases and genomic stability. In this Cell Science at a Glance and the accompanying poster, we give a brief overview of this complex system. In addition, we discuss the pathogenic basis for VCP/p97-associated diseases and then highlight in more detail new exciting links to the translational stress response and RNA biology that further underscore the significance of the VCP/p97 system.
© 2014. Published by The Company of Biologists Ltd.

Entities:  

Keywords:  ALS; Cdc48; IBMPFD; Ubiquitin; VCP; p97

Mesh:

Substances:

Year:  2014        PMID: 25146396      PMCID: PMC4163641          DOI: 10.1242/jcs.093831

Source DB:  PubMed          Journal:  J Cell Sci        ISSN: 0021-9533            Impact factor:   5.285


  102 in total

1.  Late-onset autosomal dominant limb girdle muscular dystrophy and Paget's disease of bone unlinked to the VCP gene locus.

Authors:  Michael Kottlors; Olaf Moske-Eick; Angela Huebner; Sabine Krause; Klaus Mueller; Wolfram Kress; Ralf Schwarzwald; Antje Bornemann; Verena Haug; Markus Heitzer; Janbernd Kirschner
Journal:  J Neurol Sci       Date:  2010-02-08       Impact factor: 3.181

2.  Nuclear transport defects and nuclear envelope alterations are associated with mutation of the Saccharomyces cerevisiae NPL4 gene.

Authors:  C DeHoratius; P A Silver
Journal:  Mol Biol Cell       Date:  1996-11       Impact factor: 4.138

Review 3.  p97 and close encounters of every kind: a brief review.

Authors:  I Dreveny; V E Pye; F Beuron; L C Briggs; R L Isaacson; S J Matthews; C McKeown; X Yuan; X Zhang; P S Freemont
Journal:  Biochem Soc Trans       Date:  2004-11       Impact factor: 5.407

Review 4.  Diverse functions with a common regulator: ubiquitin takes command of an AAA ATPase.

Authors:  Yihong Ye
Journal:  J Struct Biol       Date:  2006-02-20       Impact factor: 2.867

5.  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.

Authors:  M J Kovach; B Waggoner; S M Leal; D Gelber; R Khardori; M A Levenstien; C A Shanks; G Gregg; M T Al-Lozi; T Miller; W Rakowicz; G Lopate; J Florence; G Glosser; Z Simmons; J C Morris; M P Whyte; A Pestronk; V E Kimonis
Journal:  Mol Genet Metab       Date:  2001-12       Impact factor: 4.797

6.  Inactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in Drosophila.

Authors:  Ana Griciuc; Liviu Aron; Michel J Roux; Rüdiger Klein; Angela Giangrande; Marius Ueffing
Journal:  PLoS Genet       Date:  2010-08-26       Impact factor: 5.917

Review 7.  Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.

Authors:  Conrad C Weihl; Alan Pestronk; Virginia E Kimonis
Journal:  Neuromuscul Disord       Date:  2009-04-19       Impact factor: 4.296

8.  DVC1 (C1orf124) recruits the p97 protein segregase to sites of DNA damage.

Authors:  Emily J Davis; Christophe Lachaud; Paul Appleton; Thomas J Macartney; Inke Näthke; John Rouse
Journal:  Nat Struct Mol Biol       Date:  2012-10-07       Impact factor: 15.369

9.  Cdc48/p97 mediates UV-dependent turnover of RNA Pol II.

Authors:  Rati Verma; Robert Oania; Ruihua Fang; Geoffrey T Smith; Raymond J Deshaies
Journal:  Mol Cell       Date:  2011-01-07       Impact factor: 17.970

10.  p97 complexes as signal integration hubs.

Authors:  Hemmo Meyer
Journal:  BMC Biol       Date:  2012-06-13       Impact factor: 7.431

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  168 in total

1.  Cdc48/VCP and Endocytosis Regulate TDP-43 and FUS Toxicity and Turnover.

Authors:  Guangbo Liu; Aaron Byrd; Amanda N Warner; Fen Pei; Eman Basha; Allison Buchanan; J Ross Buchan
Journal:  Mol Cell Biol       Date:  2020-01-30       Impact factor: 4.272

2.  The Human Cytomegalovirus Transmembrane Protein pUL50 Induces Loss of VCP/p97 and Is Regulated by a Small Isoform of pUL50.

Authors:  Myoung Kyu Lee; Seokhwan Hyeon; Jin-Hyun Ahn
Journal:  J Virol       Date:  2020-06-16       Impact factor: 5.103

3.  Identification of NMS-873, an allosteric and specific p97 inhibitor, as a broad antiviral against both influenza A and B viruses.

Authors:  Jiantao Zhang; Yanmei Hu; Raymond Hau; Rami Musharrafieh; Chunlong Ma; Xu Zhou; Yin Chen; Jun Wang
Journal:  Eur J Pharm Sci       Date:  2019-03-28       Impact factor: 4.384

4.  The valosin-containing protein protects the heart against pathological Ca2+ overload by modulating Ca2+ uptake proteins.

Authors:  Shaunrick Stoll; Jing Xi; Ben Ma; Christiana Leimena; Erik J Behringer; Gangjian Qin; Hongyu Qiu
Journal:  Toxicol Sci       Date:  2019-07-31       Impact factor: 4.849

5.  p97 Composition Changes Caused by Allosteric Inhibition Are Suppressed by an On-Target Mechanism that Increases the Enzyme's ATPase Activity.

Authors:  Nam-Gu Her; Julia I Toth; Chen-Ting Ma; Yang Wei; Khatereh Motamedchaboki; Eduard Sergienko; Matthew D Petroski
Journal:  Cell Chem Biol       Date:  2016-04-21       Impact factor: 8.116

Review 6.  Small-Molecule Inhibitors of the Proteasome's Regulatory Particle.

Authors:  Christine S Muli; Wenzhi Tian; Darci J Trader
Journal:  Chembiochem       Date:  2019-05-24       Impact factor: 3.164

Review 7.  Pch2(TRIP13): controlling cell division through regulation of HORMA domains.

Authors:  Gerben Vader
Journal:  Chromosoma       Date:  2015-04-21       Impact factor: 4.316

8.  PINK1 Content in Mitochondria is Regulated by ER-Associated Degradation.

Authors:  Cristina Guardia-Laguarta; Yuhui Liu; Knut H Lauritzen; Hediye Erdjument-Bromage; Brittany Martin; Theresa C Swayne; Xuejun Jiang; Serge Przedborski
Journal:  J Neurosci       Date:  2019-07-12       Impact factor: 6.167

9.  A BAG3 chaperone complex maintains cardiomyocyte function during proteotoxic stress.

Authors:  Luke M Judge; Juan A Perez-Bermejo; Annie Truong; Alexandre Js Ribeiro; Jennie C Yoo; Christina L Jensen; Mohammad A Mandegar; Nathaniel Huebsch; Robyn M Kaake; Po-Lin So; Deepak Srivastava; Beth L Pruitt; Nevan J Krogan; Bruce R Conklin
Journal:  JCI Insight       Date:  2017-07-20

10.  VCP/p97 cooperates with YOD1, UBXD1 and PLAA to drive clearance of ruptured lysosomes by autophagy.

Authors:  Chrisovalantis Papadopoulos; Philipp Kirchner; Monika Bug; Daniel Grum; Lisa Koerver; Nina Schulze; Robert Poehler; Alina Dressler; Sven Fengler; Khalid Arhzaouy; Vanda Lux; Michael Ehrmann; Conrad C Weihl; Hemmo Meyer
Journal:  EMBO J       Date:  2016-10-17       Impact factor: 11.598

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