Genomes and Genes
Gene Symbol: VCP
Description: valosin containing protein
Alias: ALS14, CDC48, CMT2Y, HEL-220, HEL-S-70, IBMPFD, IBMPFD1, TERA, p97, transitional endoplasmic reticulum ATPase, 15S Mg(2+)-ATPase p97 subunit, TER ATPase, epididymis luminal protein 220, epididymis secretory protein Li 70, yeast Cdc48p homolog
Publications241 found, 100 shown here
- Tyrosine phosphorylation of p97 regulates transitional endoplasmic reticulum assembly in vitroC Lavoie
Department of Anatomy and Cell Biology, McGill University, Montreal, QC, H3A 2B2, Canada
Proc Natl Acad Sci U S A 97:13637-42. 2000The ATPase associated with different cellular activities family member p97, associated p47, and the t-SNARE syntaxin 5 are necessary for the cell-free reconstitution of transitional endoplasmic reticulum (tER) from starting low-density ..
- Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substratesXiaoyan Zhong
Department of Pharmacology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6084, USA
Hum Mol Genet 15:2409-20. 2006..AT3 binds VCP/p97, a key protein responsible for extracting ERAD substrates from the ER; binding is modulated by the size of the ..
- Gp78 cooperates with RMA1 in endoplasmic reticulum-associated degradation of CFTRDeltaF508Daisuke Morito
Department of Molecular and Cellular Biology, Institute for Frontier Medical Sciences, Kyoto University, Kyoto 606 8397, Japan
Mol Biol Cell 19:1328-36. 2008..Our data demonstrates that gp78 cooperates with RMA1 with E4-like activity in the ERAD of CFTRDeltaF508...
- Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing proteinGiles D J Watts
Division of Genetics, Children s Hospital Boston, 300 Longwood Avenue, Harvard Medical School, Boston, Massachusetts 02115, USA
Nat Genet 36:377-81. 2004Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD) is a dominant progressive disorder that maps to chromosome 9p21.1-p12...
- Recent advances in p97/VCP/Cdc48 cellular functionsKunitoshi Yamanaka
Department of Molecular Cell Biology, Institute of Molecular Embryology and Genetics, Kumamoto University, 2 2 1 Honjo, Kumamoto 860 0811, Japan
Biochim Biophys Acta 1823:130-7. 2012..a causative factor for inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD) and more recently as a causative factor for amyotrophic lateral sclerosis (ALS)...
- Derlin-1 is a rhomboid pseudoprotease required for the dislocation of mutant α-1 antitrypsin from the endoplasmic reticulumEthan J Greenblatt
Biophysics Program, Stanford University, Stanford, California, USA
Nat Struct Mol Biol 18:1147-52. 2011..degradation (ERAD) substrates must be dislocated across the ER membrane through a process driven by the AAA ATPase p97/VCP. Derlins recruit p97/VCP and have been proposed to be part of the dislocation machinery...
- Critical role of VCP/p97 in the pathogenesis and progression of non-small cell lung carcinomaChristopher W Valle
Department of Pediatric Respiratory Sciences, The Johns Hopkins School of Medicine, Baltimore, Maryland, USA
PLoS ONE 6:e29073. 2011Valosin-containing protein (VCP)/p97 is an AAA ATPase molecular chaperone that regulates vital cellular functions and protein-processing...
- The role of the N-domain in the ATPase activity of the mammalian AAA ATPase p97/VCPHajime Niwa
Centre for Structural Biology, Division of Molecular Biosciences, Faculty of Natural Sciences, Imperial College London, London SW7 2AZ, United Kingdom
J Biol Chem 287:8561-70. 2012p97/valosin-containing protein (VCP) is a type II ATPase associated with various cellular activities that forms a homohexamer with each protomer containing an N-terminal domain (N-domain); two ATPase domains, D1 and D2; and a disordered ..
- ATPase activity of p97/valosin-containing protein is regulated by oxidative modification of the evolutionally conserved cysteine 522 residue in Walker A motifMasakatsu Noguchi
Laboratory of Functional Biology, Kyoto University Graduate School of Biostudies and Solution Oriented Research for Science and Technology JST, Kyoto 606 8501, Japan
J Biol Chem 280:41332-41. 2005..Furthermore, a yeast mutant (delta cdc48 + cdc48[T532C]) was shown to be susceptible to oxidants-induced growth inhibition and cell death...
- Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementiaG D J Watts
Division of Genetics, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA
Clin Genet 72:420-6. 2007Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD, OMIM 167320) has recently been attributed to eight missense mutations in valosin-containing protein (VCP)...
- Identification of components of the murine histone deacetylase 6 complex: link between acetylation and ubiquitination signaling pathwaysD Seigneurin-Berny
Laboratoire de Biologie Moléculaire et Cellulaire de la Différenciation, INSERM U309, Equipe chromatine et expression des gènes, Institut Albert Bonniot, Faculte de Medecine, Domaine de la Merci, 38706 La Tronche Cedex, France
Mol Cell Biol 21:8035-44. 2001..homologues of yeast proteins known to interact with each other and involved in the ubiquitin signaling pathway: p97/VCP/Cdc48p, a homologue of yeast Cdc48p, and phospholipase A2-activating protein, a homologue of yeast UFD3 (..
- Gp78, a membrane-anchored ubiquitin ligase, associates with Insig-1 and couples sterol-regulated ubiquitination to degradation of HMG CoA reductaseBao Liang Song
Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
Mol Cell 19:829-40. 2005..In addition, gp78 couples regulated ubiquitination to degradation of reductase by binding to VCP, an ATPase that plays a key role in recognition and degradation of ERAD substrates...
- Human Fas-associated factor 1, interacting with ubiquitinated proteins and valosin-containing protein, is involved in the ubiquitin-proteasome pathwayEun Joo Song
Center for Cell Signaling Research, Division of Molecular Life Sciences and College of Pharmacy, Ewha Womans University, Seoul 120 750, South Korea
Mol Cell Biol 25:2511-24. 2005..We investigated the cellular functions of hFAF1 and found that valosin-containing protein (VCP), the multiubiquitin chain-targeting factor in the degradation of the ubiquitin-proteasome pathway, is a binding ..
- An Italian family with inclusion-body myopathy and frontotemporal dementia due to mutation in the VCP geneTeresa Gidaro
Department of Neuroscience, Catholic University, L go A Gemelli 8, 00168 Rome, Italy
Muscle Nerve 37:111-4. 2008Mutations of the valosin-containing protein gene (VCP) are responsible for autosomal-dominant hereditary inclusion-body myopathy associated with frontotemporal dementia and Paget's disease of bone. We identified the p...
- UBXD1 binds p97 through two independent binding sitesMaximilian Kern
Max Planck Institute of Biochemistry, Department of Molecular Cell Biology, Am Klopferspitz 18, 82152 Martinsried, Germany
Biochem Biophys Res Commun 380:303-7. 2009The chaperone-related p97 protein plays a central role in various cellular processes involving the ubiquitin-proteasome system...
- Transmembrane domain determinants of CD4 Downregulation by HIV-1 VpuJavier G Magadán
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
J Virol 86:757-72. 2012..of Vpu into an inactive, oligomeric form and to promote CD4 polyubiquitination and subsequent recruitment of the VCP-UFD1L-NPL4 dislocase complex...
- The ubiquitin regulatory X (UBX) domain-containing protein TUG regulates the p97 ATPase and resides at the endoplasmic reticulum-golgi intermediate compartmentCharisse M Orme
Section of Endocrinology and Metabolism, Department of Internal Medicine, University School of Medicine, New Haven, Connecticut 06520 8020, USA
J Biol Chem 287:6679-92. 2012b>p97/VCP is a hexameric ATPase that is coupled to diverse cellular processes, such as membrane fusion and proteolysis. How p97 activity is regulated is not fully understood...
- Complex of Fas-associated factor 1 (FAF1) with valosin-containing protein (VCP)-Npl4-Ufd1 and polyubiquitinated proteins promotes endoplasmic reticulum-associated degradation (ERAD)Jae Jin Lee
Center for Cell Signaling and Drug Discovery Research, College of Pharmacy, Division of Life and Pharmaceutical Sciences, Department of Bioinspired Science, Ewha Womans University, Seoul 120 750, Korea
J Biol Chem 288:6998-7011. 2013..to recruit polyubiquitinated proteins and that a C-terminal UBX domain interacts with valosin-containing protein (VCP)...
- Caveolin-1 interacts with Derlin-1 and promotes ubiquitination and degradation of cyclooxygenase-2 via collaboration with p97 complexShu Fen Chen
From the Cardiovascular Division, Institute of Biomedical Sciences, Academia Sinica, Taipei 11529, Taiwan
J Biol Chem 288:33462-9. 2013..Here, we identified Cav-1 as a key player in Derlin-1- and p97-mediated cyclooxygenase 2 (COX-2) ubiquitination and degradation...
- Structural basis for ovarian tumor domain-containing protein 1 (OTU1) binding to p97/valosin-containing protein (VCP)Su Jin Kim
From the Biomedical Research Institute, Korea Institute of Science and Technology, Seoul 136 791, Republic of Korea
J Biol Chem 289:12264-74. 2014Valosin-containing protein (VCP), also known as p97, is an AAA(+) ATPase that plays an essential role in a broad array of cellular processes including the endoplasmic reticulum-associated degradation (ERAD) pathway...
- Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutantsFei Sun
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 281:36856-63. 2006A complex involving Derlin-1 and p97 mediates the retrotranslocation and endoplasmic reticulum (ER)-associated degradation of misfolded proteins in yeast and is used by certain viruses to promote host cell protein degradation (Romisch, K...
- VCP (p97) regulates NFkappaB signaling pathway, which is important for metastasis of osteosarcoma cell lineTatsuya Asai
Department of Pathology, Osaka University Graduate School of Medicine, Suita, Osaka 565 0871, Japan
Jpn J Cancer Res 93:296-304. 2002..SSH revealed expression of the gene encoding valosin-containing protein (VCP; also known as p97) to be constitutively activated in LM8 cells, but it declined in Dunn cells when the cells ..
- Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysisEllen W Doss-Pepe
Department of Biochemistry, Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, Piscataway, NJ 08854, USA
Mol Cell Biol 23:6469-83. 2003..ataxin-3 associates with the ubiquitin- and proteasome-binding factors Rad23 and valosin-containing protein (VCP/p97), findings that support the hypothesis that ataxin-3 is a proteasome-associated factor that mediates the ..
- Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformationDalia Halawani
The Nicholas Conor Institute for Pediatric Cancer Research, 9710 Scranton Road, Suite 170, San Diego, CA 92121, USA
Mol Cell Biol 29:4484-94. 2009..dementia (hIBMPFTD) is a degenerative disorder caused by single substitutions in highly conserved residues of p97/VCP. All mutations identified thus far cluster within the NH(2) domain or the D1 ring, which are both required for ..
- Characterization of erasin (UBXD2): a new ER protein that promotes ER-associated protein degradationJing Liang
Graduate Program in Molecular Medicine, and Institute for Neurodegenerative Diseases, University of Maryland Biotechnology Institute, 725 West Lombard Street, Baltimore, MD 21201, USA
J Cell Sci 119:4011-24. 2006Ubiquitin regulator-X (UBX) is a discrete protein domain that binds p97/valosin-containing protein (VCP), a molecular chaperone involved in diverse cell processes, including endoplasmic-reticulum-associated protein degradation (ERAD)...
- HIV-1 Vpu neutralizes the antiviral factor Tetherin/BST-2 by binding it and directing its beta-TrCP2-dependent degradationBastien Mangeat
Department of Dermatology and Venereology, University Hospitals and Medical School of Geneva, University of Geneva, Switzerland
PLoS Pathog 5:e1000574. 2009..Identification of tetherin binding to Vpu provides a potential novel target for the development of drugs aimed at inhibiting HIV-1 replication...
- AAA ATPase p97/valosin-containing protein interacts with gp78, a ubiquitin ligase for endoplasmic reticulum-associated degradationXiaoyan Zhong
Medical Biotechnology Center, University of Maryland Biotechnology Institute, Baltimore, Maryland 21201, USA
J Biol Chem 279:45676-84. 2004..AAA ATPase (ATPase associated with various cellular activities) p97/valosin-containing protein (VCP) subsequently dislodges the ubiquitinated proteins from the ER and chaperones them to the cytosol, where they ..
- Inclusion body myopathy and Paget disease is linked to a novel mutation in the VCP geneD Haubenberger
Department of Neurology, Medical University of Vienna, Vienna, Austria
Neurology 65:1304-5. 2005..to be associated with hereditary inclusion body myopathy, Paget disease of the bone, and frontotemporal dementia (IBMPFD)...
- Unsaturated fatty acids inhibit proteasomal degradation of Insig-1 at a postubiquitination stepJoon No Lee
Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
J Biol Chem 283:33772-83. 2008..The current study provides a molecular mechanism for regulation of proteasome-mediated ER protein degradation at a postubiquitination step...
- Valosin containing protein associated inclusion body myopathy: abnormal vacuolization, autophagy and cell fusion in myoblastsJouni Vesa
Department of Pediatrics, Division of Genetics and Metabolism, University of California, Irvine, CA 92868, USA
Neuromuscul Disord 19:766-72. 2009Inclusion body myopathy associated with Paget's disease and frontotemporal dementia (IBMPFD) is caused by mutations in the valosin containing protein (VCP) gene...
- Dual role of ancient ubiquitous protein 1 (AUP1) in lipid droplet accumulation and endoplasmic reticulum (ER) protein quality controlElizabeth J Klemm
Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02142, USA
J Biol Chem 286:37602-14. 2011..These findings indicate a possible connection between ER protein quality control and lipid droplets...
- An NSF-like ATPase, p97, and NSF mediate cisternal regrowth from mitotic Golgi fragmentsC Rabouille
Cell Biology Laboratory, Imperial Cancer Research Fund, London, England
Cell 82:905-14. 1995..Pretreatment with NEM or salt washing inhibited regrowth, but this could be restored either by p97, an NSF-like ATPase, or by NSF together with SNAPs and p115, a vesicle docking protein...
- Identification of the cell cycle regulator VCP (p97/CDC48) as a substrate of the band 4.1-related protein-tyrosine phosphatase PTPH1S H Zhang
Cold Spring Harbor Laboratory, Cold Spring Harbor, New York 11724, USA
J Biol Chem 274:17806-12. 1999..primarily a 97-kDa tyrosine-phosphorylated protein, which was determined to be VCP (also named p97 or yeast CDC48), from various cell lysates in vitro...
- Elevated expression of valosin-containing protein (p97) in hepatocellular carcinoma is correlated with increased incidence of tumor recurrenceShinji Yamamoto
Departments of Surgery and Clinical Oncology, and Pathology, Osaka University Graduate School of Medicine, Osaka, Japan
J Clin Oncol 21:447-52. 2003Valosin-containing protein (VCP; also known as p97) has been shown to be associated with antiapoptotic function and metastasis via activation of the nuclear factor-kappaB signaling pathway...
- Valosin-containing protein phosphorylation at Ser784 in response to DNA damageMark Livingstone
Cell Signaling Technology, Inc, Beverly, Massachusetts, USA
Cancer Res 65:7533-40. 2005..These include histone H2AX and DNA-PK itself. We identify here valosin-containing protein (VCP) as a novel substrate of DNA-PK and other PIKK family members...
- Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulatorJ Michael Younger
Department of Cell and Developmental Biology, UNC Chapel Hill School of Medicine, University of North Carolina, Chapel Hill, NC 27599, USA
Cell 126:571-82. 2006..Thus, the RMA1 and CHIP E3 ubiquitin ligases act sequentially in ER membrane and cytosol to monitor the folding status of CFTR and CFTR Delta F508...
- Valosin-containing protein (p97) is a regulator of endoplasmic reticulum stress and of the degradation of N-end rule and ubiquitin-fusion degradation pathway substrates in mammalian cellsCezary Wojcik
Department of Anatomy and Cell Biology, Indiana University School of Medicine, Evansville, IN 47712, USA
Mol Biol Cell 17:4606-18. 2006Valosin-containing protein (VCP; p97; cdc48 in yeast) is a hexameric ATPase of the AAA family (ATPases with multiple cellular activities) involved in multiple cellular functions, including degradation of proteins by the ubiquitin (Ub)-..
- The role of a novel p97/valosin-containing protein-interacting motif of gp78 in endoplasmic reticulum-associated degradationPetek Ballar
Medical Biotechnology Center, University of Maryland Biotechnology Institute, Baltimore, Maryland 21201, USA
J Biol Chem 281:35359-68. 2006..Dislocation requires a subclass of ubiquitin ligases that includes gp78 in addition to the AAA ATPase p97/VCP and its cofactor, the Ufd1-Npl4 dimer. We have previously reported that gp78 interacts directly with p97/VCP...
- Identification of SVIP as an endogenous inhibitor of endoplasmic reticulum-associated degradationPetek Ballar
University of Maryland Biotechnology Institute, 725 W Lombard Street, Baltimore, MD 21201, USA
J Biol Chem 282:33908-14. 2007..To test this hypothesis, we investigated the potential regulatory role of the small p97/VCP-interacting protein (SVIP) on the formation of the ERAD machinery that includes ubiquitin ligase gp78, AAA ATPase ..
- Inhibition of p97-dependent protein degradation by Eeyarestatin IQiuyan Wang
Laboratory of Molecular Biology, NIDDK, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 283:7445-54. 2008..the action of ubiquitinating enzymes, which polyubiquitinate substrates emerging from the ER lumen, and of the p97-Ufd1-Npl4 ATPase complex, which hydrolyzes ATP to dislocate polyubiquitinated substrates into the cytosol...
- An endoplasmic reticulum (ER) membrane complex composed of SPFH1 and SPFH2 mediates the ER-associated degradation of inositol 1,4,5-trisphosphate receptorsMargaret M P Pearce
Departments of Pharmacology and Biochemistry and Molecular Biology, SUNY Upstate Medical University, Syracuse, New York 13210, USA
J Biol Chem 284:10433-45. 2009..We propose that this novel SPFH1/2 complex is a recognition factor that targets IP(3)Rs and perhaps other substrates for ERAD...
- Clearance of Rhodopsin(P23H) aggregates requires the ERAD effector VCPAna Griciuc
Department of Protein Science, Helmholtz Zentrum Muenchen German Research Center for Environmental Health GmbH, 85764 Neuherberg, Germany
Biochim Biophys Acta 1803:424-34. 2010..mammalian cell cultures, we provide the first evidence that misfolded Rh(P23H) is a substrate of the ERAD effector VCP, an ATP-dependent chaperone that extracts misfolded proteins from the ER and escorts them for proteasomal ..
- Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCPHui Yang
Center for Biomedical Engineering and Technology, University of Maryland, Baltimore, Maryland, United States of America
PLoS ONE 5:e8905. 2010..competitively reduces polyubiquitinated protein binding to gp78 and also sterically blocks gp78 interaction of p97/VCP, a molecular chaperone that is essential for ERAD...
- Valosin-containing protein (VCP) in novel feedback machinery between abnormal protein accumulation and transcriptional suppressionMasaaki Koike
Laboratory of Functional Biology, Kyoto University Graduate School of Biostudies and Solution Oriented Research for Science and Technology, Kyoto 606 8501, Japan
J Biol Chem 285:21736-49. 2010..Genetic and biochemical analyses indicate that valosin-containing protein (VCP) is a crucial molecule in the pathogenesis of human neurodegenerative disorders...
- Multilayered mechanism of CD4 downregulation by HIV-1 Vpu involving distinct ER retention and ERAD targeting stepsJavier G Magadán
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America
PLoS Pathog 6:e1000869. 2010..we find that CD4 degradation induced by Vpu is dependent on a key component of the ERAD machinery, the VCP-UFD1L-NPL4 complex, as well as on SCF(beta-TrCP)-dependent ubiquitination of the CD4 cytosolic tail on lysine and ..
- Cellular functions of Ufd2 and Ufd3 in proteasomal protein degradation depend on Cdc48 bindingStefanie Böhm
Department of Biochemistry, Biocenter, University of Wurzburg, Am Hubland, 97074 Wurzburg, Germany
Mol Cell Biol 31:1528-39. 2011The chaperone-related AAA ATPase Cdc48 (p97/VCP in higher eukaryotes) segregates ubiquitylated proteins for subsequent degradation by the 26S proteasome or for nonproteolytic fates...
- DNA damage modulates nucleolar interaction of the Werner protein with the AAA ATPase p97/VCPJuneth Joaquin Partridge
Laboratory of Cell Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
Mol Biol Cell 14:4221-9. 2003We report a novel nucleolar interaction between the AAA ATPase p97/VCP and the Werner protein (WRNp), a member of the RecQ helicase family...
- Pre-B-cell leukemia transcription factor 1 regulates expression of valosin-containing protein, a gene involved in cancer growthYing Qiu
Department of Pathology, Medical School of Tongji University, Shanghai, China
Am J Pathol 170:152-9. 2007Valosin-containing protein (VCP) is involved in a wide variety of cellular functions. Our previous studies showed that the enhanced expression of VCP in cancer cells correlated with invasion and metastasis of cancers...
- Proteomic characterization of protein phosphatase 1 complexes in ischemia-reperfusion and ischemic toleranceCristina Cid
Department of Investigation, Hospital Ramon y Cajal, Madrid, Spain
Proteomics 7:3207-18. 2007..It supports a potential role of PP1 in IR stress and as a target of the endogenous protective mechanisms induced by IT...
- Derlin-1 and p97/valosin-containing protein mediate the endoplasmic reticulum-associated degradation of human V2 vasopressin receptorsIsabel Schwieger
Leibniz Institut fur Molekulare Pharmakologie FMP, Campus Buch, Robert Rossle Str 10, 13125 Berlin, Germany
Mol Pharmacol 73:697-708. 2008..for the first time that V2R mutants with different misfolded domains are able to coprecipitate the ERAD components p97/valosin-containing protein, Derlin-1 and the 26S proteasome regulatory subunit 7...
- Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disordersShinsuke Ishigaki
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466 8500, Japan
J Biol Chem 279:51376-85. 2004..Here we report that Valosin-containing protein (VCP) directly binds to Dorfin and that VCP ATPase activity profoundly contributes to the E3 activity of Dorfin...
- VCP, a weak ATPase involved in multiple cellular events, interacts physically with BRCA1 in the nucleus of living cellsH Zhang
Department of Pathology, University of Pennsylvania School of Medicine, Philadelphia 19104 6082, USA
DNA Cell Biol 19:253-63. 2000..Here, we demonstrate that the BRCA1 protein physically associates with valosin-containing protein (VCP), a member of the ATPases associated with a variety of cellular activities (AAA) superfamily...
- Expression of valosin-containing protein in colorectal carcinomas as a predictor for disease recurrence and prognosisShinji Yamamoto
Department of Surgery and Clinical Oncology, Osaka University Graduate School of Medicine, Osaka, Japan
Clin Cancer Res 10:651-7. 2004Valosin-containing protein (VCP or p97) is associated with antiapoptotic function and metastasis via activation of the nuclear factor-kappaB signaling pathway...
- Differential regulation of CFTRDeltaF508 degradation by ubiquitin ligases gp78 and Hrd1Petek Ballar
Ege University, Faculty of Pharmacy, Izmir, 35100, Turkey
Int J Biochem Cell Biol 42:167-73. 2010..gp78 facilitates the degradation of CFTRDeltaF508 by enhancing both its ubiquitination and interaction with p97/VCP. SVIP, which is the inhibitor of gp78, causes accumulation of CFTRDeltaF508...
- Valosin-containing protein, VCP, is a ubiquitous clathrin-binding proteinI T Pleasure
Department of Pharmacology, Thomas Jefferson University, Philadelphia, Pennsylvania 19107
Nature 365:459-62. 1993..The 100K protein has been identified as valosin-containing protein (VCP; ref. 1), an early substrate for tyrosine phosphorylation on T-cell receptor activation...
- Syntaxin 5 is a common component of the NSF- and p97-mediated reassembly pathways of Golgi cisternae from mitotic Golgi fragments in vitroC Rabouille
Cell Biology Laboratory, Imperial Cancer Research Fund, London, United Kingdom
Cell 92:603-10. 1998A cell-free system that mimics the reassembly of Golgi stacks at the end of mitosis requires two ATPases, NSF and p97, to rebuild Golgi cisternae...
- Clinical and molecular studies in a unique family with autosomal dominant limb-girdle muscular dystrophy and Paget disease of boneV E Kimonis
Department of Pediatircs, Southern Illinois University School of Medicine, Springfield 62794 9658, USA
Genet Med 2:232-41. 2000..To characterize the clinical features and perform linkage analysis of candidate loci in a large Illinois family with autosomal dominant limb-girdle muscular dystrophy (LGMD) and Paget disease of bone (PDB)...
- Valosin-containing protein is a multi-ubiquitin chain-targeting factor required in ubiquitin-proteasome degradationR M Dai
Intramural Research Support Program, SAIC Frederick, National Cancer Institute at Frederick, Frederick, Maryland 21702, USA
Nat Cell Biol 3:740-4. 2001..We have shown previously that valosin-containing protein (VCP) physically and functionally targets the ubiquitinated nuclear factor kappaB inhibitor, IkappaBalpha to the ..
- Functional ATPase activity of p97/valosin-containing protein (VCP) is required for the quality control of endoplasmic reticulum in neuronally differentiated mammalian PC12 cellsTaeko Kobayashi
Graduate School of Biostudies, Kyoto University, Kyoto, 606 8501, Japan
J Biol Chem 277:47358-65. 2002..We previously identified p97/valosin-containing protein (VCP), an AAA ATPase with two conserved ATPase domains (D1 and D2), as an interacting partner of the Machado-Joseph ..
- VCIP135, a novel essential factor for p97/p47-mediated membrane fusion, is required for Golgi and ER assembly in vivoKeiji Uchiyama
Cambridge Institute for Medical Research, University of Cambridge, Cambridge CB2 2XY, United Kingdom
J Cell Biol 159:855-66. 2002NSF and p97 are ATPases required for the heterotypic fusion of transport vesicles with their target membranes and the homotypic fusion of organelles...
- SVIP is a novel VCP/p97-interacting protein whose expression causes cell vacuolationMasami Nagahama
School of Life Science, Tokyo University of Pharmacy and Life Science, Hachioji, Tokyo 192 0392, Japan
Mol Biol Cell 14:262-73. 2003b>VCP/p97 is involved in a variety of cellular processes, including membrane fusion and ubiquitin-dependent protein degradation. It has been suggested that adaptor proteins such as p47 and Ufd1p confer functional versatility to VCP/p97...
- D1 ring is stable and nucleotide-independent, whereas D2 ring undergoes major conformational changes during the ATPase cycle of p97-VCPQing Wang
Basic Research Laboratory, Science Applications International Corporation Frederick, National Cancer Institute, National Institutes of Health, Frederick, Maryland 21702, USA
J Biol Chem 278:32784-93. 2003The 97-kDa valosin-containing protein (p97-VCP) belongs to the AAA (ATPases associated with various cellular activities) family and acts as a molecular chaperone in diverse cellular events, including ubiquitinproteasome-mediated ..
- A membrane protein complex mediates retro-translocation from the ER lumen into the cytosolYihong Ye
Howard Hughes Medical Institute and Department of Cell Biology, Harvard Medical School, 240 Longwood Avenue, Boston, Massachusetts 02115, USA
Nature 429:841-7. 2004..recognition of a substrate in the ER lumen and its subsequent movement through the membrane by the cytosolic p97 ATPase. Here we identify a p97-interacting membrane protein complex in the mammalian ER that links these two events...
- The AAA ATPase p97/VCP interacts with its alternative co-factors, Ufd1-Npl4 and p47, through a common bipartite binding mechanismRoland M Bruderer
Swiss Federal School of Technology, Institute of Biochemistry, ETH Honggerberg HPM, Zurich 8093, Switzerland
J Biol Chem 279:49609-16. 2004The AAA ATPase p97/VCP forms complexes with different adapters to fulfill distinct cellular functions...
- Valosin-containing protein (p97) and Ki-67 expression is a useful marker in detecting malignant behavior of pancreatic endocrine neoplasmsShinji Yamamoto
Department of Surgery and Clinical Oncology, Osaka University Graduate School of Medicine, Yamadaoka, Suita City, Japan
Oncology 66:468-75. 2004To determine the prognostic value of valosin-containing protein (VCP) expression and the Ki-67 labeling index (LI) in pancreatic endocrine neoplasms (PENs), the present analysis was employed.
- Mutant valosin-containing protein causes a novel type of frontotemporal dementiaRolf Schroder
Department of Neurology, University Hospital Bonn, Sigmund Freud Strasse 25, 53105 Bonn, Germany
Ann Neurol 57:457-61. 2005..myopathy and frontotemporal dementia who harbors a heterozygous R155C missense mutation residing in the N-terminal CDC48 domain of VCP, which is involved in ubiquitin binding...
- The ubiquitin-domain protein HERP forms a complex with components of the endoplasmic reticulum associated degradation pathwayAndrea Schulze
Charite, Universitaetsmedizin Berlin, Campus Charite Mitte, Institut für Biochemie Monbijoustr 2, 10117 Berlin, Germany
J Mol Biol 354:1021-7. 2005..ubiquitin domain protein (UDP) HERP, the ubiquitin protein ligase HRD1, as well as the retro-translocation factors p97, Derlin-1 and VIMP...
- Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradationYukako Oda
Department of Molecular and Cellular Biology Institute for Frontier Medical Sciences, Graduate School of Science, Kyoto University, Sakyo ku, Kyoto 606 8502, Japan
J Cell Biol 172:383-93. 2006..Derlin-2 and -3 are associated with EDEM and p97, a cytosolic ATPase responsible for extraction of ERAD substrates...
- An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesisAnnett Boeddrich
Department of Neuroproteomics, Max Delbrueck Center for Molecular Medicine MDC, Berlin, Germany
EMBO J 25:1547-58. 2006..protein ataxin-3 (Atx-3) serves as a recognition site for the interaction with the molecular chaperone VCP. Through this interaction, VCP modulates the fibrillogenesis of pathogenic forms of Atx-3 in a concentration-..
- The valosin-containing protein (VCP) is a target of Akt signaling required for cell survivalFranck Vandermoere
ERI 8 INSERM, Growth factor signaling in breast cancer Functional proteomics, University of Sciences and Technologies Lille, 59655 Villeneuve d Ascq, France
J Biol Chem 281:14307-13. 2006..We show, using a proteomics-based approach, that the valosin-containing protein (VCP), a member of the AAA (ATPases associated with a variety of cellular activities) family, is a target of Akt ..
- Endoplasmic reticulum vacuolization and valosin-containing protein relocalization result from simultaneous hsp90 inhibition by geldanamycin and proteasome inhibition by velcadeEdward G Mimnaugh
Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, NIH, 9000 Rockville Pike, Building 10, Room 1 5940, Bethesda, MD 20892 1107, USA
Mol Cancer Res 4:667-81. 2006..lumen are cleared by ER--associated protein degradation, a sequential process requiring valosin-containing protein (VCP)-dependent retrotranslocation followed by ubiquitination and proteasomal proteolysis...
- Mutations in p97/VCP induce unfolding activityAndrea Rothballer
Max Planck Institute of Biochemistry, Department of Molecular Structural Biology, Am Klopferspitz 18, 82152 Martinsried, Germany
FEBS Lett 581:1197-201. 2007..In contrast, none of the members of the eukaryotic p97/VCP protein family has an aromatic residue in the D1 pore...
- Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathyJake B Guinto
Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 422 Curie Blvd, 605B Stellar Chance Building, Philadelphia, PA, 19104 6140, USA
Acta Neuropathol 114:55-61. 2007Frontotemporal dementia with inclusion body myopathy and Paget's disease of bone (IBMPFD) is a rare, autosomal dominant disorder caused by mutations in the gene valosin-containing protein (VCP)...
- Analysis of Npl4 deletion mutants in mammalian cells unravels new Ufd1-interacting motifs and suggests a regulatory role of Npl4 in ERADAgnieszka Lass
Department of Anatomy and Cell Biology, Indiana University School of Medicine, 8600 University Boulevard, Evansville, IN 47712, USA
Exp Cell Res 314:2715-23. 2008Npl4 is a 67 kDa protein forming a stable heterodimer with Ufd1, which in turn binds the ubiquitous p97/VCP ATPase...
- Ubxd1 is a novel co-factor of the human p97 ATPaseLouise Madsen
Department of Biology, University of Copenhagen, Universitetsparken 13, DK 2100 Copenhagen Ø, Denmark
Int J Biochem Cell Biol 40:2927-42. 2008The AAA ATPase complex known as p97 or VCP in mammals and Cdc48 in yeast is connected to a multitude of cellular pathways, including membrane fusion, protein folding, protein degradation and activation of membrane-bound transcription ..
- SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteinsBritta Mueller
Whitehead Institute for Biomedical Research, 9 Cambridge Center, Cambridge, MA 02142, USA
Proc Natl Acad Sci U S A 105:12325-30. 2008..We identified AUP1, UBXD8, UBC6e, and OS9 as functionally important components of this degradation complex in mammalian cells, as confirmed by mutagenesis and dominant negative versions of these proteins...
- UBXD7 binds multiple ubiquitin ligases and implicates p97 in HIF1alpha turnoverGabriela Alexandru
Division of Biology, California Institute of Technology, 1200 East California Boulevard, Pasadena, CA 91125, USA
Cell 134:804-16. 2008b>p97 is an ATP-dependent chaperone that plays an important role in endoplasmic reticulum-associated degradation but whose connections to turnover of soluble proteins remain sparse...
- Evolutionary divergence of valosin-containing protein/cell division cycle protein 48 binding interactions among endoplasmic reticulum-associated degradation proteinsGiacomo Morreale
Laboratory of Molecular Signalling, The Babraham Institute, Cambridge, UK
FEBS J 276:1208-20. 2009..For example, valosin-containing protein (VCP) and its yeast homologue cell division cycle protein 48 (Cdc48p), which help to direct polyubiquitinated proteins ..
- A novel mutation in the VCP gene (G157R) in a German family with inclusion-body myopathy with Paget disease of bone and frontotemporal dementiaAtbin Djamshidian
Department of Neurology, Medical University of Vienna Währinger Gürtel 18 20, Vienna A 1097, Austria
Muscle Nerve 39:389-91. 2009..to cause autosomal-dominant inclusion-body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD). We report a novel missense mutation (G157R) in the N-terminal region of the VCP gene in a German family...
- Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP diseaseJeong Sun Ju
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
J Cell Biol 187:875-88. 2009..protein (VCP) cause inclusion body myopathy (IBM), Paget's disease of the bone, and frontotemporal dementia (IBMPFD)...
- Imbalances in p97 co-factor interactions in human proteinopathyVanesa Fernández-Sáiz
Department of Molecular Cell Biology, Max Planck Institute of Biochemistry, Am Klopferspitz 18, 82152 Martinsried, Germany
EMBO Rep 11:479-85. 2010..in p97 cause the disorder inclusion body myopathy with Paget disease of bone and frontotemporal dementia (IBMPFD)...
- The p97 ATPase dislocates MHC class I heavy chain in US2-expressing cells via a Ufd1-Npl4-independent mechanismNia Soetandyo
Laboratory of Molecular Biology, NIDDK, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 285:32352-9. 2010..The retrotranslocation also involves the p97 ATPase...
- Valosin-containing protein cleavage by granzyme K accelerates an endoplasmic reticulum stress leading to caspase-independent cytotoxicity of target tumor cellsYuming Guo
Key Laboratory of Infection and Immunity, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China
J Immunol 185:5348-59. 2010..In this study, we found the valosin-containing protein (VCP) is a physiological substrate of GzmK...
- Exome sequencing reveals VCP mutations as a cause of familial ALSJanel O Johnson
Neuromuscular Diseases Research Group, Laboratory of Neurogenetics, Porter Neuroscience Building, NIA, NIH, Bethesda, MD 20892, USA
Neuron 68:857-64. 2010..previously been identified in families with Inclusion Body Myopathy, Paget disease, and Frontotemporal Dementia (IBMPFD)...
- The complexities of p97 function in health and diseaseEli Chapman
Department of Molecular Biology, The Scripps Research Institute, Skaggs Molecular Biology Building, 10596 Torrey Pines Road, Rm 203, La Jolla, CA 92037, USA
Mol Biosyst 7:700-10. 2011b>p97 is a homohexameric, toroidal machine that harnesses the energy of ATP binding and hydrolysis to effect structural reorganization of a diverse and primarily uncharacterized set of substrate proteins...
- The p97 ATPase associates with EEA1 to regulate the size of early endosomesHarish N Ramanathan
Laboratory of Molecular Biology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA
Cell Res 22:346-59. 2012The AAA (ATPase-associated with various cellular activities) ATPase p97 acts on diverse substrate proteins to partake in various cellular processes such as membrane fusion and endoplasmic reticulum-associated degradation (ERAD)...
- Cdc48: a power machine in protein degradationAlexandra Stolz
Institute for Biochemistry, University of Stuttgart, Pfaffenwaldring 55, 70569 Stuttgart, Germany
Trends Biochem Sci 36:515-23. 2011b>Cdc48 is an essential, highly prominent ATP driven machine in eukaryotic cells...
- Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutationsDanilo Ritz
Centre for Medical Biotechnology, University of Duisburg Essen, 45117 Essen, Germany
Nat Cell Biol 13:1116-23. 2011The AAA-ATPase VCP (also known as p97) cooperates with distinct cofactors to process ubiquitylated proteins in different cellular pathways...
- The tissue-specific Rep8/UBXD6 tethers p97 to the endoplasmic reticulum membrane for degradation of misfolded proteinsLouise Madsen
Department of Biology, University of Copenhagen, Copenhagen, Denmark
PLoS ONE 6:e25061. 2011The protein known as p97 or VCP in mammals and Cdc48 in yeast is a versatile ATPase complex involved in several biological functions including membrane fusion, protein folding, and activation of membrane-bound transcription factors...
- Characterization of the Asian myopathy patients with VCP mutationsZ Shi
Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry NCNP, Kodaira, Tokyo, Japan
Eur J Neurol 19:501-9. 2012..VCP) gene are known to cause inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) and familial amyotrophic lateral sclerosis (ALS)...
- Distinct conformations of the protein complex p97-Ufd1-Npl4 revealed by electron cryomicroscopyCecilia Bebeacua
Centre for Structural Biology and Centre for Biomolecular Electron Microscopy, Division of Molecular Biosciences, Imperial College London, London SW7 2AZ, United Kingdom
Proc Natl Acad Sci U S A 109:1098-103. 2012b>p97 is a key regulator of numerous cellular pathways and associates with ubiquitin-binding adaptors to remodel ubiquitin-modified substrate proteins...
- Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin systemHemmo Meyer
Centre for Medical Biotechnology, Faculty of Biology, University of Duisburg Essen, 45117 Essen, Germany
Nat Cell Biol 14:117-23. 2012The ATP-driven chaperone valosin-containing protein (VCP)/p97 governs critical steps in ubiquitin-dependent protein quality control and intracellular signalling pathways...
- Increased expression of valosin-containing protein in the skin of patients with amyotrophic lateral sclerosisHiroaki Ishikawa
Department of Neurology, Teikyo University Chiba Medical Center, 3426 3 Anesaki, Ichihara, Chiba 299 0111, Japan
J Clin Neurosci 19:522-6. 2012Valosin-containing protein (VCP) may have a pivotal role in ubiquitin-dependent protein degradation and is implicated in the pathogenesis of neurodegenerative diseases...
- Interprotomer motion-transmission mechanism for the hexameric AAA ATPase p97Guangtao Li
Department of Biochemistry and Cell Biology, Stony Brook University, Stony Brook, NY 11794 5215, USA
Proc Natl Acad Sci U S A 109:3737-41. 2012..The essential and highly abundant hexameric AAA ATPase p97 is perhaps the best studied AAA protein, playing an essential role in various important cellular activities...
- Phenotypic variability in three families with valosin-containing protein mutationS Spina
Department of Pathology and Laboratory Medicine, Indiana Alzheimer Disease Center, Indiana University School of Medicine, Indianapolis, IN 46202, USA
Eur J Neurol 20:251-8. 2013The phenotype of IBMPFD [inclusion body myopathy with Paget's disease of the bone and frontotemporal dementia (FTD)] associated with valosin-containing protein (VCP) mutation is described in three families.
- Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3Mario N Laco
Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal
PLoS ONE 7:e43563. 2012..with two proteins previously described to interact with ataxin-3, hHR23A and valosin-containing protein (VCP/p97). We confirmed ataxin-3 affinity for both hHR23A and VCP/p97...
- A ubiquitin-like domain recruits an oligomeric chaperone to a retrotranslocation complex in endoplasmic reticulum-associated degradationYue Xu
Laboratory of Molecular Biology, NIDDK, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 288:18068-76. 2013..We propose that simultaneous association with multiple ERAD factors helps to anchor a disordered chaperone oligomer to the site of retrotranslocation to prevent protein aggregation in ERAD. ..
- Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic miceCarlos J Rodriguez-Ortiz
School of Natural Sciences, University of California, Merced, California 95343, USA
Am J Pathol 183:504-15. 2013..dominant disease called inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD)...
- Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body diseaseFumiaki Mori
Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki, Japan
Neuropathology 33:637-44. 2013..body myopathy with Paget's disease and frontotemporal dementia and familial amyotrophic lateral sclerosis (fALS; ALS14), both of which are characterized by trans-activation response DNA protein 43 (TDP-43)-positive neuronal ..
- Involvement of valosin-containing protein, an ATPase Co-purified with IkappaBalpha and 26 S proteasome, in ubiquitin-proteasome-mediated degradation of IkappaBalphaR M Dai
Intramural Research Support Program, SAIC Frederick, NCI Frederick Cancer Research and Development Center, Frederick, Maryland 21702, USA
J Biol Chem 273:3562-73. 1998..We identify valosin-containing protein (VCP), an AAA (ATPases associated with a variety of cellular activities) family member, that co-precipitates with ..
- Multiprotein complexes that link dislocation, ubiquitination, and extraction of misfolded proteins from the endoplasmic reticulum membraneBrendan N Lilley
Department of Pathology, Harvard Medical School, 77 Avenue Louis Pasteur, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 102:14296-301. 2005..Derlin-1 acts in concert with the AAA ATPase p97 to remove dislocation substrate proteins from the ER membrane, but it is unknown whether other factors aid Derlin-1 ..
- Conrad C Weihl; Fiscal Year: 2016..further by the identification of mutations in the protein p97/VCP that cause the autosomal dominant syndrome, IBMPFD, IBM associated with paget's disease of the bone and frontotemporal dementia (FTD)...
- Mariusz Karbowski; Fiscal Year: 2015..Furthermore, we also found that AAA- ATPase p97, a critical protein for the endoplasmic reticulum associated degradation (ERAD) pathway, is essential for both ..
- James A Olzmann; Fiscal Year: 2015..identify a metabolically regulated LD ubiquitination pathway that involves UBXD8-mediated recruitment of p97/VCP and regulation LD turnover catalyzed by ATGL, the rate-limiting enzyme in lipolysis...
- Defining the role of ubiquitination in the regulation of lipid droplet functionJames A Olzmann; Fiscal Year: 2013..findings identify a metabolically regulated LD ubiquitination pathway that involves UBXD8-mediated recruitment of p97/VCP and regulation LD turnover catalyzed by ATGL, the rate-limiting enzyme in lipolysis...
- High-fat diet rescues lethality of homozygous knock-in R155H VCP myopathic miceVIRGINIA EUNICE KIMONIS; Fiscal Year: 2013DESCRIPTION (provided by applicant): Our laboratory was the first to identify VCP mutations as a cause of hereditary inclusion body myopathy, Paget's disease of bone, frontotemporal dementia and amyotrophic lateral sclerosis, and ..
- Translational Studies in VCP Inclusion Body Myopathy with Paget Disease of BoneVirginia Kimonis; Fiscal Year: 2009..inclusion body myopathy (h-IBM), Paget disease of bone (PDB) and/or frontotemporal dementia (FTD), also called IBMPFD was recognized as a distinct clinical syndrome in 2000 by Kimonis et al...
- Changcheng Song; Fiscal Year: 2014..Valosin-containing protein (VCP/p97), a molecular chaperone, lies at the heart of proteostasis to unfold ubiquitinated proteins for proteasomal ..
- Cellular Mechanisms of Aggregation of Abnormal ProteinsMichael Y Sherman; Fiscal Year: 2012..element for the P-region aggresome- targeting signal, (c) a yeast 14-3-3 protein Bmh1 and components of the Cdc48/VCP-Ufd1-Nlp4 complex play an essential role in aggresome formation...
- The role of p97 in protein degradation during aging and diseaseEUGENE DROKHLYANSKY; Fiscal Year: 2013..Previous studies support that p97 (known as VCP in metazoans and Cdc48 in yeast) is important for the degradation of damaged and misfolded proteins possibly by preventing their ..
- Ubiquitin and Cell Cycle Regulation of Golgi Membrane DynamicsYanzhuang Wang; Fiscal Year: 2013..Reassembly is mediated by two ATPases (NSF and p97) that fuse the membranes...
- Exploring mechanisms of TDP-43 aggregation and disaggregationJames Shorter; Fiscal Year: 2010..We are particularly interested in how two AAA+ ATPases, Hsp104 and p97, might antagonize or reverse TDP-43 aggregation...
- Mechanisms of VCP/p97 and Proteasomal pathway in COPD pathogenesisNeeraj Vij; Fiscal Year: 2010..protein levels of AAA (ATPase associated with diverse cellular activities) molecular chaperone and UPR modulator, p97/VCP (Valosin containing protein), are significantly upregulated in COPD peripheral lungs of smokers as compared to ..
- Alex B Lentsch; Fiscal Year: 2014..The cytoplasmic chaperone, valosin- containing protein (VCP), is known to bind to ubiquitinylated proteins, including IkBa, and facilitate their recruitment to the proteasome...
- Jianping Jin; Fiscal Year: 2016..Recent studies in our lab suggest that a valosin-containing protein (VCP) and its cofactor, UFD1L, are involved in post-ubiquitinational regulation of I?B[unreadable]...
- Understanding and targeting Nrf1-mediated proteasome recovery pathway in cancerSENTHIL KUMAR RADHAKRISHNAN; Fiscal Year: 2012..Specific Aims during the independent phase are designed to understand the role of p97/VCP and its co-factors in Nrf1 activation and to identify novel chemical inhibitors of the Nrf1-mediated proteasome ..
- KENNETH R TETER; Fiscal Year: 2016..Most ERAD substrates are extracted from the ER through a mechanism involving the cytosolic AAA ATPase p97. However, p97 appears to play a minimal role in CTA1 translocation...
- Role of the Sec61 Translocon in EGF Receptor Trafficking and SignalingGRAHAM F CARPENTER; Fiscal Year: 2012..This includes HSP70 and p97, an ATPase that is tyrosine phosphorylated following the addition of EGF...
- Nuclear Membrane Fusion in Xenopus Egg ExtractsMartin W Hetzer; Fiscal Year: 2010..generated new information about the two major regulators of nuclear assembly, the GTPase Ran and the AAA-ATPase p97. Our specific aims are: 1...
- SVIP and CMT1AJun Li; Fiscal Year: 2013..SVIP interacts with Valosin-Containing Protein (VCP;also called p97), a highly conserved ATPase that is ubiquitously distributed...
- Smallpox vaccine and vaccinia complement control proteinSTUART ISAACS; Fiscal Year: 2009..We hypothesize that the vaccinia complement control protein (VCP) is a virulence factor with both a central role in protecting the virus from innate immunity and an ..
- Conrad C Weihl; Fiscal Year: 2016..One age associated muscle disorder is due to mutations in valosin containing protein (VCP) which causes IBMPFD or inclusion body myopathy (IBM) associated with Paget's disease of the bone (PDB) and fronto-temporal ..
- Fen Biao Gao; Fiscal Year: 2016..Indeed, several genes, including CHMP2B, VCP, TDP-43, FUS, Ubiquilin 2, and C9ORF72, have been implicated in the molecular pathogenesis of both diseases...
- CYTOPLASMIC FACTORS IN NUCLEAR PROTEIN IMPORTStephen Adam; Fiscal Year: 2000..Cytoplasmic assembly of a receptor complex composed of two transport factors, p97 and the NLS receptor is believed to precede docking of the complex at the nuclear pore...
- Masashi Kitazawa; Fiscal Year: 2014..disorder called inclusion body myopathy associated with Paget disease ofthe bone and frontotemporal dementia (IBMPFD)...
- Characterizing the Role of VCP in Paget's Disease of the BoneGiles Watts; Fiscal Year: 2009..combination of hereditary inclusion body myopathy, Paget disease of bone and frontotemporal dementia, now called IBMPFD (MIM 605382). I have recently identified the gene for IBMPFD as VCP...
- HPV-16 and head and neck cancerThomas Haugen; Fiscal Year: 2013..of the cervix and the head and neck contain and express the viral E6-E7 oncogenic region from a promoter at nt 97 (P97)...
- GENETIC BASES FOR DISEASES OF THE RANK SIGNALING PATHWAYSTEVEN R MUMM; Fiscal Year: 2013..candidate genes in the OPG/RANKL/RANK/NF-?B signaling pathway for disease-causing mutations, including SQSTM1, VCP, TRAF6, aPKC, NIK, and others...
- Dihydrodiol dehydrogenase mediates cisplatin resistanceHenry Simpkins; Fiscal Year: 2007..We propose to continue this work by (a) transfection of non-ovarian cell lines, including Tera-2 (germ cell tumors), A431(cervical carcinoma cells), HTB56 (lung adenocarcinoma ceils) and H69 (small cell lung ..
- Influence of Heme on Hepatic Cytochrome P450 Synthesis and DegradationMaria Correia; Fiscal Year: 2007..ER extraction requires ATP hydrolysis and thus could involve either the p97 AAA ATPase or the proteasomal 19S AAA ATPases...
- BIGDATA: Mid-Scale: DA : Collaborative Research Big Tensor Mining TheoryChristos Faloutsos; Fiscal Year: 2013..or action-like verbs ('run')? We propose three research thrusts: i) Motivating Applications: Real-world tera-scale applications, including 'read-the-web', NeuroSemanticslarge recommendation systems ii) New theory ..
- MEMBRANE FUSION ATPASES AND THE GOLGI APPARATUSAyano Satoh; Fiscal Year: 2007..The last granting period focused on the reassembly of Golgi cisternae that is catalyzed by two ATPases, NSF and p97. NSF was shown to have an activity additional to its well-characterized role in unraveling SNARE complexes...
- Study of Interactions of VCP with Complement ComponentsKrishna Murthy; Fiscal Year: 2007..Vaccinia Complement Protein (VCP) was one of the first such mimics to be discovered and characterized...
- New anti-complement therapy to reduce reperfusion injuryClaudio Maldonado; Fiscal Year: 2005..to optimize the display of a modified complement blocking protein named vaccinia virus complement control protein (VCP) on the surface of endothelial cells, and show that it can reduce complement induced damage after cells are exposed ..
- POXVIRUS SCR-CONTAINING PROTEINS AS THERAPEUTIC TARGETSSTUART ISAACS; Fiscal Year: 2003..The vaccinia complement-control protein (VCP) and the extracellular enveloped virus (EEV)-specific B5R protein both contain short consensus repeat (SCR) units ..
- AB 3730XL, 96 Cap DNA AnalyzerMitchell Sogin; Fiscal Year: 2005..The equipment to be purchased includes an Applied Biosystems 3730XL DNA Analyzer and a 3.4 tera-byte data storage system based upon Apple Computer's Xserve RAID and Xserve G5 architecture...
- The role of ubiquitination in Legionella pneumophila infection and disease.ALEXANDER ENSMINGER; Fiscal Year: 2009..vacuole (LCV) upon infection and a downstream component of the host ubiquitin-mediated proteolytic pathway, Cdc48/p97 is required for efficient intracellular growth of the bacteria. Additionally, the L...
- EPIDERMAL TRANS-ACTING FACTORS IN SKIN DEVELOPMENTRenata Polakowska; Fiscal Year: 1993..genes and a keratinocyte-dependent (KD) enhancer sequence element of the human papilloma virus-16 (HPV-16) p97 promoter. Viral KD-enhancer activation is restricted to keratinocytes...
- MOSQUITO OOCYTES - MECHANISM OF PROTEIN ACCUMULATIONAlexander Raikhel; Fiscal Year: 2001..In addition, I propose to study the receptor for vitellogenic carboxypeptidase (VCP), another important mosquito yolk protein precursor...
- HUMAN PAPILLOMAVIRUS-16 REGULATION IN CERVICAL CANCERLUBOMIR TUREK; Fiscal Year: 1991..and viral regulatory factors at the transcriptional promoter of the candidate E6-E7 oncogene region of HPV-16, P97, in transfection and DNA binding studies. Based on our recent data (Cripe et aL, 1987, ref...
- Role of Gab2 in Mast Cell Function and OntogenyHaihua Gu; Fiscal Year: 2006..long-term goal of this grant proposal is to understand the mechanism by which the novel scaffolding adapter Gab2/p97 regulates the biological responses of hematopoietic cells...
- AAA ATPase p97/VCP and Inclusion Body MyopathyCONRAD WEIHL; Fiscal Year: 2009..in p97/VCP cause the autosomal dominantly syndrome HIBM, Paget's Disease and frontotemporal dementia (IBMPFD)...
- Validation of a Human In Vitro Vaginal Irritation TestSeyoum Ayehunie; Fiscal Year: 2007..agents contained within spermicides, medicaments, microbicides, feminine hygiene and other vaginal care products (VCP) can cause inflammation or tissue damage and thus endanger the health of women...
- Evaluation and Application of an Adapted Measure of Inspection Time of Children wSeth Warschausky; Fiscal Year: 2009..The first group consists of children who communicate verbally (VCP) and have functional activity levels classified as GMFCS I-III...
- MECHANISM OF PLATELET ACTIVATION ON MATRIX PROTEINSBeatrice Haimovich; Fiscal Year: 2004..and/or aggregated platelets: a-actinin, vinculin, and a lO1-kD protein (pplOl) that is identical or related to VCP (Valosin-containing protein). Recently we reported that a-actinin is tyrosine phosphorylated in spread platelets...
- ROLE OF BAG2 AND SHP2 IN HEMATOPOIETIC SIGNALLINGBENJAMIN NEEL; Fiscal Year: 2004..During the initial funding period, we identified, purified and cloned p97. p97 is a relative of Gab1 and Drosophila Dos, a regulator of the fly homolog of SHP-2; thus, we renamed p97 as ..
- Targeting JAK-3 with WHI-P97 for Treatment of AsthmaPeter Samuel; Fiscal Year: 2003..designed Janus kinase 3 (JAK3) inhibitor 4-(3',5'-dibromo-4'-hydroxyphenyl)- anmino-6,7-dirnethoxyquinazoline (WHI-P97) exhibits potent in vivo biologic activity in a mouse model of allergic asthma at nontoxic dose levels...
- Modeling TDP-43 Dysfunction in vivoZuoshang Xu; Fiscal Year: 2009..protein tau (MAPT), charged multivesicular body protein 2B (CHMP2B), vasolin-containing protein (VCP) and progranulin (PGRN). How these mutant genes cause neuronal degeneration is not known...
- HPV16-induced TGF-Beta resistance in human keratinocytesKIM CREEK; Fiscal Year: 2006..In addition, the molecular basis for the marked inhibition of HPV16 P97 promoter activity by TGF-beta will be investigated...
- PROTEOLYTIC SIGNALING BY POLYUBIQUITIN CHAINSMichael Matunis; Fiscal Year: 2006..rate and pathway of proteasome-dependent degradation and b) how an extraproteasomal chaperone complex containing Cdc48/VCP functions in the degradation of ubiquitin-fused substrates...
- THE MOLECULAR NATURE OF TUMOR ANTIGENSRICHARD WOODBURY; Fiscal Year: 1980..The other two melanoma-associated antigens are proteins with molecular weights of approximately 97,000 (p97) and 210,000 (p210), respectively...
- Single Molecule Studies of SNARE-Induced Vesicle FusionAxel T Brunger; Fiscal Year: 2010..Such a system could serve as an efficient model system for novel drug discovery. ..
- GENE CAUSING PAGET & LIMB-GIRDLE MUSCULAR DYSTROPHYVirginia Kimonis; Fiscal Year: 2003..Delineation of the genetic component responsible for the LGMD/PDB phenotype should promise similar insight and facilitate in the design of novel treatment protocols for the two disorders. ..
- Genetic basis of myopathy with Paget disease of boneVirginia Kimonis; Fiscal Year: 2008..of disorders such as limb girdle muscular dystrophy and amyotropic lateral sclerosis has been categorized as IBMPFD (MIM 605382)...
- Characterization of Familial Myopathy & Paget DiseaseVirginia Kimonis; Fiscal Year: 2005..of disorders such as limb girdle muscular dystrophy and amyotropic lateral sclerosis has been categorized as IBMPFD (MIM 605382)...
- INHIBITION OF OSTEOCLAST ACTIVITY BY OIP-1/HSCALSAKAMURI REDDY; Fiscal Year: 2008..abstract_text> ..
- Pathogenic determinants in the SIV envelope transmembrane cytoplasmic domainJames Hoxie; Fiscal Year: 2007..abstract_text> ..
- Parkin mediated Neural Dysfunction in DrosophilaLeo Pallanck; Fiscal Year: 2004..Results from this work should clarify the relationship between parkin dysfunction and neurodegeneration, and possibly reveal strategies for treatment of Parkinson's disease. ..
- The role of hMLH1-hMRE11 in DNA mismatch repairChengtao Her; Fiscal Year: 2007..abstract_text> ..
- GENETIC DISSECTION OF DENDRITE DEVELOPMENTYUH JAN; Fiscal Year: 2009..The Core programs identified from this work will contribute to the understanding and eventual treatment of human neurological diseases many of which have pathology in dendrites. ..
- Mechanisms of Tau-Based NeurodegenerationMark Forman; Fiscal Year: 2005..This proposal will also facilitate my transition from a trainee to a fully independent experimental neuropathologist. ..
- High-Throughput Campaign to Identify Reversible Small Molecule Inhibitor of p97/CRaymond Deshaies; Fiscal Year: 2008unreadable] DESCRIPTION (provided by applicant): p97 is an essential protein that plays a critical role in a number of different cellular processes...
- Gene Expression in Inflammatory MyopathiesSteven Greenberg; Fiscal Year: 2005..This work may provide further diagnostic approaches to these disorders and contribute to the understanding of their pathogenesis. ..
- HIV-2 Env Variable Loop Deletions as HIV-1 VaccinesGeorge Lin; Fiscal Year: 2006..Two Specific Aims are proposed: #1) engineer HIV-1 converting mutations into wildtype HIV-2/vcp Env and functional, hypervariable loop deleted derivatives and assess for retention of functionality and #2) ..
- Proteomic analyses of Drosophila Parkinson's disease modelLeo Pallanck; Fiscal Year: 2007..This work should clarify the biological role of Parkin and will serve as a foundation for future hypothesis-driven investigation of parkin pathogenesis. [unreadable] [unreadable]..
- Pathogenic Determinants of SIV Envelope GlycoproteinsJames Hoxie; Fiscal Year: 2006..Collectively these Aims provide novel approaches for structure-based alterations in the SIV TM that will impact on pathogenesis and the host immune response. ..
- Cell Mechanisms of Abnormal Protein AggregationMichael Sherman; Fiscal Year: 2007..Exploration of fundamental mechanisms of protein aggregation that we undertake in this project will help to understand the nature of several neurological disorders. ..
- Mtb Proteasome-Associated Factors and VirulenceKATERINA DARWIN; Fiscal Year: 2007..Taken together it will be important to develop faster acting drugs to new targets in Mtb in order to better treat tuberculosis in the future. ..