The complexities of p97 function in health and disease.

No abstract is available for this paper.

• Publication year

  2011

• Venue

  Molecular Biosystems

• Publication date

  2011-03-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1039/c0mb00176gPMID 21152665PMCID PMC3050576
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• TDP-43 Mediates Degeneration in a Novel Drosophila Model of Disease Caused by Mutations in VCP/p97

  2010cited by this paper
• Emerging role of Alzheimer's disease-associated ubiquilin-1 in protein aggregation.

  2010cited by this paper
• The p97 ATPase Dislocates MHC Class I Heavy Chain in US2-expressing Cells via a Ufd1-Npl4-independent Mechanism*

  2010cited by this paper
• A Novel Histone Deacetylase Inhibitor Exhibits Antitumor Activity via Apoptosis Induction, F-Actin Disruption and Gene Acetylation in Lung Cancer

  2010cited by this paper
• Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy.

  2010cited by this paper
• Structural and functional implications of phosphorylation and acetylation in the regulation of the AAA+ protein p97.

  2010cited by this paper
• ATP-bound form of the D1 AAA domain inhibits an essential function of Cdc48p/p97.

  2010cited by this paper
• Huntingtin Interacts with the Cue Domain of gp78 and Inhibits gp78 Binding to Ubiquitin and p97/VCP

  2010cited by this paper
• A novel ATP‐dependent conformation in p97 N–D1 fragment revealed by crystal structures of disease‐related mutants

  2010cited by this paper
• VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

  2010cited by this paper
• Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.

  2010cited by this paper
• p97/VCP at the intersection of the autophagy and the ubiquitin proteasome system

  2010cited by this paper
• 2-Anilino-4-aryl-1,3-thiazole inhibitors of valosin-containing protein (VCP or p97).

  2010cited by this paper
• New ATPase regulators--p97 goes to the PUB.

  2009cited by this paper
• Crystal structures of asymmetric ClpX hexamers reveal nucleotide-dependent motions in a AAA+ protein-unfolding machine

  2009cited by this paper
• Structural changes in intermediate filament networks alter the activity of insulin‐degrading enzyme

  2009cited by this paper
• VCP Mutations Causing Frontotemporal Lobar Degeneration Disrupt Localization of TDP-43 and Induce Cell Death*

  2009cited by this paper
• Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.

  2009cited by this paper
• A conserved unfoldase activity for the p97 AAA-ATPase in proteasomal degradation.

  2009cited by this paper
• Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

  2009cited by this paper
• Hereditary Inclusion Body Myopathy-Linked p97/VCP Mutations in the NH2 Domain and the D1 Ring Modulate p97/VCP ATPase Activity and D2 Ring Conformation

  2009cited by this paper
• Motor Mechanism for Protein Threading through Hsp104

  2009cited by this paper
• Eeyarestatin I inhibits Sec61-mediated protein translocation at the endoplasmic reticulum

  2009cited by this paper
• An Armadillo motif in Ufd3 interacts with Cdc48 and is involved in ubiquitin homeostasis and protein degradation

  2009cited by this paper
• ERAD inhibitors integrate ER stress with an epigenetic mechanism to activate BH3-only protein NOXA in cancer cells

  2009cited by this paper
• Both ATPase Domains of ClpA Are Critical for Processing of Stable Protein Structures

  2009cited by this paper
• Insights into adaptor binding to the AAA protein p97.

  2008cited by this paper
• ALS and FTLD: two faces of TDP‐43 proteinopathy

  2008cited by this paper
• Roles of VCP in human neurodegenerative disorders.

  2008cited by this paper
• Impaired Protein Aggregate Handling and Clearance Underlie the Pathogenesis of p97/VCP-associated Disease*

  2008cited by this paper
• Structure of the A20 OTU domain and mechanistic insights into deubiquitination.

  2008cited by this paper
• Improved structures of full-length p97, an AAA ATPase: implications for mechanisms of nucleotide-dependent conformational change.

  2008cited by this paper
• UBX domain proteins: major regulators of the AAA ATPase Cdc48/p97

  2008cited by this paper
• UBXD7 binds multiple ubiquitin ligases and implicates p97 in HIF1alpha turnover.

  2008cited by this paper
• Response to: “Comment on ‘Production of multivalent protein binders using a self‐trimerization collagen‐like peptide scaffold’”

  2008cited by this paper
• Mechanisms of Cdc48/VCP-mediated cell death: from yeast apoptosis to human disease.

  2008cited by this paper
• Inhibition of p97-dependent Protein Degradation by Eeyarestatin I*

  2008cited by this paper
• Analysis of Nucleotide Binding to P97 Reveals the Properties of a Tandem AAA Hexameric ATPase

  2008cited by this paper
• Detailed Structural Insights into the p97-Npl4-Ufd1 Interface*

  2007cited by this paper
• The AAA+ superfamily--a myriad of motions.

  2007cited by this paper
• Studies on peptide:N-glycanase–p97 interaction suggest that p97 phosphorylation modulates endoplasmic reticulum-associated degradation

  2007cited by this paper
• Dual enzymatic properties of the cytoplasmic peptide: N-glycanase in C. elegans.

  2007cited by this paper
• HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

  2007cited by this paper
• TDP-43: a novel neurodegenerative proteinopathy.

  2007cited by this paper
• Atypical AAA+ Subunit Packing Creates an Expanded Cavity for Disaggregation by the Protein-Remodeling Factor Hsp104

  2007cited by this paper
• Cdc48 (p97): a "molecular gearbox" in the ubiquitin pathway?

  2007cited by this paper
• Identification of SVIP as an Endogenous Inhibitor of Endoplasmic Reticulum-associated Degradation*

  2007cited by this paper
• Structure and function of the yeast U-box-containing ubiquitin ligase Ufd2p

  2007cited by this paper
• TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease

  2007cited by this paper
• Involvement of valosin‐containing protein (VCP)/p97 in the formation and clearance of abnormal protein aggregates

  2007cited by this paper
• Frontotemporal Lobar Degeneration: Current Concepts in the Light of Recent Advances

  2007cited by this paper
• Ubiquitin receptors and ERAD: a network of pathways to the proteasome.

  2007cited by this paper
• Structural insights into the p97-Ufd1-Npl4 complex

  2007cited by this paper
• Molecular chaperones regulate p53 and suppress senescence programs

  2007cited by this paper
• The activity of a human endoplasmic reticulum-associated degradation E3, gp78, requires its Cue domain, RING finger, and an E2-binding site.

  2006cited by this paper
• Characterization of erasin (UBXD2): a new ER protein that promotes ER-associated protein degradation

  2006cited by this paper
• Going through the motions: the ATPase cycle of p97.

  2006cited by this paper
• Conformational changes in the AAA ATPase p97–p47 adaptor complex

  2006cited by this paper
• Ataxin-3 binds VCP/p97 and regulates retrotranslocation of ERAD substrates.

  2006cited by this paper
• The PUB Domain Functions as a p97 Binding Module in Human Peptide N-Glycanase*

  2006cited by this paper
• Evolutionary relationships and structural mechanisms of AAA+ proteins.

  2006cited by this paper
• Doa1 Is a Cdc48 Adapter That Possesses a Novel Ubiquitin Binding Domain

  2006cited by this paper
• Functional division of substrate processing cofactors of the ubiquitin-selective Cdc48 chaperone.

  2006cited by this paper
• p97: The cell's molecular purgatory?

  2006cited by this paper
• The AAA ATPase p97 links peptide N-glycanase to the endoplasmic reticulum-associated E3 ligase autocrine motility factor receptor.

  2006cited by this paper
• Characterization of Ubiquitinated Intraneuronal Inclusions in a Novel Belgian Frontotemporal Lobar Degeneration Family

  2006cited by this paper
• Diverse functions with a common regulator: ubiquitin takes command of an AAA ATPase.

  2006cited by this paper
• Transcriptional regulation of the methuselah gene by dorsal protein in Drosophila melanogaster.

  2006cited by this paper
• Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation.

  2006cited by this paper
• Central pore residues mediate the p97/VCP activity required for ERAD.

  2006cited by this paper
• The Role of a Novel p97/Valosin-containing Protein-interacting Motif of gp78 in Endoplasmic Reticulum-associated Degradation*

  2006cited by this paper
• An arginine/lysine‐rich motif is crucial for VCP/p97‐mediated modulation of ataxin‐3 fibrillogenesis

  2006cited by this paper
• Genetic Prognostic Index Influences Patient Outcome for Node-Positive Breast Cancer

  2006cited by this paper
• Molecular biology of amyotrophic lateral sclerosis: insights from genetics

  2006cited by this paper
• Deubiquitinating function of ataxin-3: insights from the solution structure of the Josephin domain.

  2005cited by this paper
• Loops in the central channel of ClpA chaperone mediate protein binding, unfolding, and translocation.

  2005cited by this paper
• VAT, the Thermoplasma Homolog of Mammalian p97/VCP, Is an N Domain-regulated Protein Unfoldase*

  2005cited by this paper
• Multiple modes of interaction of the deglycosylation enzyme, mouse peptide N-glycanase, with the proteasome.

  2005cited by this paper
• Increased Expression of Valosin-Containing Protein (p97) Is Correlated With Disease Recurrence in Follicular Thyroid Cancer

  2005cited by this paper
• A series of ubiquitin binding factors connects CDC48/p97 to substrate multiubiquitylation and proteasomal targeting.

  2005cited by this paper
• AAA+ proteins: have engine, will work

  2005cited by this paper
• Inclusion body myopathy and Paget disease is linked to a novel mutation in the VCP gene

  2005cited by this paper
• The ubiquitin-proteasome system in Creutzfeldt-Jakob and Alzheimer disease: intracellular redistribution of components correlates with neuronal vulnerability.

  2005cited by this paper
• Golgi reassembly after mitosis: the AAA family meets the ubiquitin family.

  2005cited by this paper
• Human Fas-Associated Factor 1, Interacting with Ubiquitinated Proteins and Valosin-Containing Protein, Is Involved in the Ubiquitin-Proteasome Pathway

  2005cited by this paper
• Recruitment of the p97 ATPase and ubiquitin ligases to the site of retrotranslocation at the endoplasmic reticulum membrane.

  2005cited by this paper
• Conformational changes of p97 during nucleotide hydrolysis determined by small-angle X-Ray scattering.

  2005cited by this paper
• Golgi reassembly after mitosis: the AAA family meets the ubiquitin family.

  2005cited by this paper
• The Ubx2 and Ubx3 cofactors direct Cdc48 activity to proteolytic and nonproteolytic ubiquitin-dependent processes.

  2004cited by this paper
• VCIP135 acts as a deubiquitinating enzyme during p97–p47-mediated reassembly of mitotic Golgi fragments

  2004cited by this paper
• Hsp104 Catalyzes Formation and Elimination of Self-Replicating Sup35 Prion Conformers

  2004cited by this paper
• Structural basis of the interaction between the AAA ATPase p97/VCP and its adaptor protein p47

  2004cited by this paper
• Increased Expression of Valosin-Containing Protein (p97) is Associated With Lymph Node Metastasis and Prognosis of Pancreatic Ductal Adenocarcinoma

  2004cited by this paper
• The AAA ATPase p97/VCP Interacts with Its Alternative Co-factors, Ufd1-Npl4 and p47, through a Common Bipartite Binding Mechanism*

  2004cited by this paper
• Thermotolerance requires refolding of aggregated proteins by substrate translocation through the central pore of ClpB.

  2004cited by this paper
• A membrane protein complex mediates retro-translocation from the ER lumen into the cytosol

  2004cited by this paper
• Expression level of valosin-containing protein (VCP) as a prognostic marker for gingival squamous cell carcinoma.

  2004cited by this paper
• Shp1 and Ubx2 are adaptors of Cdc48 involved in ubiquitin‐dependent protein degradation

  2004cited by this paper
• Expression of Valosin-Containing Protein in Colorectal Carcinomas as a Predictor for Disease Recurrence and Prognosis

  2004cited by this paper
• Atomic snapshots of an RNA packaging motor reveal conformational changes linking ATP hydrolysis to RNA translocation.

  2004cited by this paper

• ATP-competitive inhibitors for cancer treatment – kinases and the world beyond

  2025cites this paper
• Harnessing p97/VCP: A Transformative AAA+ ATPase Target for Next-Generation Cancer Therapeutics

  2025cites this paper
• Muscle Biopsy Findings in Valosin-Containing Protein Multisystem Proteinopathy

  2025cites this paper
• Discovery of novel pyrimidine molecules containing boronic acid as VCP/p97 Inhibitors.

  2021cites this paper
• Macromolecular Protein Complexes III: Structure and Function

  2021cites this paper
• Structure, Dynamics and Function of the 26S Proteasome.

  2021cites this paper
• Structural and Functional Analysis of Disease-Linked p97 ATPase Mutant Complexes

  2021cites this paper
• AAA+ ATPases in Protein Degradation: Structures, Functions and Mechanisms

  2020cites this paper
• Synthesis and In Vitro Evaluation of 2-[3-(2-Aminoethyl)-1H-indol-1-yl]-N-benzylquinazolin-4-amine as a Novel p97/VCP Inhibitor Lead Capable of Inducing Apoptosis in Cancer Cells

  2020cites this paper
• Pharmacophore modeling, atom-based 3D-QSAR and molecular docking studies on N-benzylpyrimidin-4-amine derivatives as VCP/p97 inhibitors

  2020cites this paper
• Investigation of protein stability as underlying molecular pathology in 21-hydroxylase deficiency

  2019cites this paper
• Target Protein-Oriented Natural Product Isolation Methods

  2019cites this paper
• Genome-Wide Identification and Transcriptional Expression of the METTL21C Gene Family in Chicken

  2019cites this paper
• Structure and Function of the AAA+ ATPase p97, a Key Player in Protein Homeostasis.

  2019cites this paper
• Proteomic profiling of VCP substrates links VCP to K6‐linked ubiquitylation and c‐Myc function

  2018cites this paper
• Depletion of Ubiquilin induces an augmentation in soluble ubiquitinated Drosophila TDP-43 to drive neurotoxicity in the fly.

  2018cites this paper
• Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease?

  2017cites this paper
• Ubiquitin- and ATP-dependent unfoldase activity of P97/VCP•NPLOC4•UFD1L is enhanced by a mutation that causes multisystem proteinopathy

  2017influential citation
• Mouse Mammary Tumor Virus Signal Peptide Uses a Novel p97-Dependent and Derlin-Independent Retrotranslocation Mechanism To Escape Proteasomal Degradation

  2017cites this paper
• Targeting protein quality control pathways in breast cancer

  2017cites this paper
• Association between SNPs and haplotypes in the METTL21C gene and peak bone mineral density and body composition in Chinese male nuclear families

  2017cites this paper
• Molecular Mechanism of Substrate Processing by the Cdc48 ATPase Complex.

  2017cites this paper
• The Interplay of Cofactor Interactions and Post-translational Modifications in the Regulation of the AAA+ ATPase p97

  2017cites this paper
• Interaction between the AAA+ ATPase p97 and its cofactor ataxin3 in health and disease: Nucleotide-induced conformational changes regulate cofactor binding

  2017cites this paper
• Ubiquitin-and ATP-dependent unfoldase activity of 1 P 97 / VCP

  2017influential citation
• p97 Negatively Regulates NRF2 by Extracting Ubiquitylated NRF2 from the KEAP1-CUL3 E3 Complex

  2017cites this paper
• Oxaspirol B with p97 Inhibitory Activity and Other Oxaspirols from Lecythophora sp. FL1375 and FL1031, Endolichenic Fungi Inhabiting Parmotrema tinctorum and Cladonia evansii.

  2016cites this paper
• Protein-Protein Interface and Disease: Perspective from Biomolecular Networks.

  2016cites this paper
• Dual Unnatural Amino Acid Incorporation and Click‐Chemistry Labeling to Enable Single‐Molecule FRET Studies of p97 Folding

  2016cites this paper
• Valosin‐containing Protein is a Target of 5′‐l Fuligocandin B and Enhances TRAIL Resistance in Cancer Cells

  2016cites this paper
• Selective inhibition of p97 by chlorinated analogues of dehydrocurvularin.

  2016cites this paper
• Evaluating p97 Inhibitor Analogues for Potency against p97–p37 and p97–Npl4–Ufd1 Complexes

  2016cites this paper
• Pathogenic Mutations in the Valosin-containing Protein/p97(VCP) N-domain Inhibit the SUMOylation of VCP and Lead to Impaired Stress Response*

  2016cites this paper
• Targeting p97 to Disrupt Protein Homeostasis in Cancer

  2016cites this paper
• HRD1 partners in endoplasmic reticulum-associated degradation

  2015cites this paper
• The disruption of proteostasis in neurodegenerative disorders

  2015cites this paper
• Proteomic Analysis Reveals Aberrant O-GlcNAcylation of Extracellular Proteins from Breast Cancer Cell Secretion.

  2015cites this paper
• Origin and Functional Evolution of the Cdc48/p97/VCP AAA+ Protein Unfolding and Remodeling Machine.

  2015cites this paper
• Arginine methylation of HSP70 regulates retinoid acid-mediated RARβ2 gene activation

  2015cites this paper
• Inhibitors of the AAA+ Chaperone p97

  2015influential citation
• Evaluating p97 Inhibitor Analogues for Their Domain Selectivity and Potency against the p97–p47 Complex

  2015cites this paper
• Lysine Methylation of the Valosin-Containing Protein (VCP) Is Dispensable for Development and Survival of Mice

  2015cites this paper
• Withaferin A Analogs That Target the AAA+ Chaperone p97.

  2015cites this paper
• Evaluation of valosin containing protein (P97) as a cancer biomaker in canine lymphomas

  2015cites this paper
• Create and preserve: proteostasis in development and aging is governed by Cdc48/p97/VCP.

  2014cites this paper
• Structural Insights into the Interaction between AAA+ ATPase p97/VCP and its Substrate Processing Factor, OTU1

  2014cites this paper
• The p97-FAF1 Protein Complex Reveals a Common Mode of p97 Adaptor Binding

  2014cites this paper
• Functional Chromatography Reveals Three Natural Products that Target the Same Protein with Distinct Mechanisms of Action

  2014cites this paper
• Inter-ring rotations of AAA ATPase p97 revealed by electron cryomicroscopy

  2014cites this paper
• Mechanism of transient binding and release of substrate protein during the allosteric cycle of the p97 nanomachine.

  2013cites this paper
• Interaction between Salt-inducible Kinase 2 (SIK2) and p97/Valosin-containing Protein (VCP) Regulates Endoplasmic Reticulum (ER)-associated Protein Degradation in Mammalian Cells*

  2013cites this paper
• A unique IBMPFD-related P97/VCP mutation with differential binding pattern and subcellular localization.

  2013cites this paper
• Assembly of the heteromeric Pex 1 p-Pex 6 p-complex of the peroxisomal matrix protein import machinery

  2013cites this paper
• Perturbations to the Ubiquitin Conjugate Proteome in Yeast Δubx Mutants Identify Ubx2 as a Regulator of Membrane Lipid Composition*

  2013cites this paper
• UBXN1 interferes with Rig-I-like receptor-mediated antiviral immune response by targeting MAVS.

  2013influential citation
• Chromatin-associated protein degradation: influence of p97/VCP on key factors in the DNA damage response

  2013influential citation
• Ubiquitination of the N-terminal Region of Caveolin-1 Regulates Endosomal Sorting by the VCP/p97 AAA-ATPase*

  2013cites this paper
• Molecular mechanism of curcumin on the suppression of cholesterol accumulation in macrophage foam cells and atherosclerosis.

  2012cites this paper
• NMR characterization of the interaction between the PUB domain of peptide:N‐glycanase and ubiquitin‐like domain of HR23

  2012cites this paper
• Simulations of the p97 complex suggest novel conformational states of hydrolysis intermediates

  2012cites this paper
• Interprotomer motion-transmission mechanism for the hexameric AAA ATPase p97

  2012cites this paper
• Lysine methylation of VCP by a member of a novel human protein methyltransferase family

  2012cites this paper
• Dynamic flexibility of the ATPase p97 is important for its interprotomer motion transmission

  2012cites this paper
• The AAA-type ATPases Pex1p and Pex6p and their role in peroxisomal matrix protein import in Saccharomyces cerevisiae.

  2012cites this paper
• Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system

  2012cites this paper
• Expanding into new markets--VCP/p97 in endocytosis and autophagy.

  2012cites this paper
• Neurodegeneration as a consequence of failed mitochondrial maintenance

  2012cites this paper
• Growing sphere of influence: Cdc48/p97 orchestrates ubiquitin-dependent extraction from chromatin.

  2012cites this paper
• Recent advances in p97/VCP/Cdc48 cellular functions.

  2012cites this paper
• The Cdc48 machine in endoplasmic reticulum associated protein degradation.

  2012cites this paper
• Protein folding and quality control in the ER.

  2011cites this paper
• Requirements for Mouse Mammary Tumor Virus Rem Signal Peptide Processing and Function

  2011cites this paper
• Endoplasmic reticulum associated protein degradation (ERAD): the function of Dfm1 and other novel components of the pathway

  2011cites this paper
• ATP-bound form of the D1 AAA domain inhibits an essential function of Cdc48p/p97.

  2010cites this paper