Valosin Containing Protein

A highly-conserved AAA ATPase that functions in the biogenesis of the transitional ENDOPLASMIC RETICULUM and fragmentation and reassembly of the GOLGI APPARATUS during MITOSIS. It also functions in a complex with UFD1L and NPLOC4 proteins to export misfolded ubiquitinated proteins from the endoplasmic reticulum and outer mitochondrial membrane to the cytoplasm for degradation by the PROTEASOME and also plays a role in AUTOPHAGY of ubiquitinated proteins. It occurs in neuronal INCLUSION BODIES from patients with AMYOTROPHIC LATERAL SCLEROSIS and LEWY BODIES from PARKINSON DISEASE patients.

Also Known As:

CDC48 Protein; Cell Cycle Protein CDC48; Cell Cycle Protein CDC48p; Valosin-Containing Protein; Valosine-Containing Protein; p97 Valosin-Containing Protein; Valosin-Containing Protein, p97; Valosine Containing Protein; p97 Valosin Containing Protein

Networked: 318 relevant articles (9 outcomes, 18 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Kimonis, Virginia E: 27 articles (01/2021 - 04/2004)
2.	Nalbandian, Angèle: 16 articles (02/2017 - 10/2010)
3.	Kimonis, Virginia: 14 articles (06/2022 - 03/2005)
4.	Aozasa, Katsuyuki: 13 articles (07/2009 - 02/2003)
5.	Tomita, Yasuhiko: 13 articles (07/2009 - 02/2003)
6.	Weihl, Conrad C: 11 articles (01/2020 - 01/2006)
7.	Llewellyn, Katrina J: 11 articles (02/2017 - 01/2012)
8.	Hoshida, Yoshihiko: 9 articles (11/2005 - 02/2003)
9.	Yamamoto, Shinji: 9 articles (11/2005 - 02/2003)
10.	Kakizuka, Akira: 8 articles (01/2022 - 12/2002)

Related Diseases

1.	Muscular Diseases (Myopathy) 01/01/2018 - "Loss of the novel Vcp (valosin containing protein) interactor Washc4 interferes with autophagy-mediated proteostasis in striated muscle and leads to myopathy in vivo." 03/15/2013 - "mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy." 11/01/2009 - "Valosin containing protein associated inclusion body myopathy: abnormal vacuolization, autophagy and cell fusion in myoblasts." 11/03/2022 - "Variants in the valosin-containing protein (VCP) gene were identified as one of the causes for inclusion body myopathy associated with Paget disease of the bone and frontotemporal dementia (FTD). " 12/01/2019 - "Valosin-containing protein-related myopathy and Meige syndrome: Just a coincidence or not?"
2.	Frontotemporal Dementia (Semantic Dementia) 11/03/2022 - "Valosin-containing protein Asp395Gly mutation in a patient with frontotemporal dementia: a case report." 01/01/2021 - "A Novel Mutation (D395A) in Valosin-Containing Protein Gene Is Associated With Early Onset Frontotemporal Dementia in an Italian Family." 03/01/2005 - "Mutant valosin-containing protein causes a novel type of frontotemporal dementia." 01/01/2023 - "Case report: Frontotemporal dementia and amyotrophic lateral sclerosis caused by a missense variant (p.Arg89Trp) in the valosin-containing protein gene." 11/03/2022 - "Variants in the valosin-containing protein (VCP) gene were identified as one of the causes for inclusion body myopathy associated with Paget disease of the bone and frontotemporal dementia (FTD). "
3.	Proteostasis Deficiencies 01/01/2018 - "Genotype-phenotype study in patients with valosin-containing protein mutations associated with multisystem proteinopathy." 10/01/2023 - "Therapeutic developments for valosin-containing protein mediated multisystem proteinopathy." 10/01/2023 - "Clinical Classification of Variants in the Valosin-Containing Protein Gene Associated With Multisystem Proteinopathy." 12/01/2022 - "One proteinopathy called multisystem proteinopathy (MSP) is associated with dominant mutations in Valosin Containing Protein (VCP). " 01/01/2022 - "Development of a standard of care for patients with valosin-containing protein associated multisystem proteinopathy."
4.	Inclusion Body Myositis 01/01/2023 - "The rationale for this study was based on reports that valosin-containing protein (VCP) mutations are found in hereditary inclusion body myositis (IBM) and VCP was detected in rimmed vacuoles of sporadic IBM (sIBM) muscle biopsies. " 10/01/2007 - "Nuclear localization of valosin-containing protein in normal muscle and muscle affected by inclusion-body myositis." 09/01/2016 - "Necrotizing autoimmune neuropathies, the co-occurrence of inclusion body myositis and a form of T-cell leukemia are discussed as are valosin-containing protein (VCP)-opathy and bone health in patients with dystrophinopathy. " 06/01/2012 - "Since valosin-containing protein mutations were reported as a cause of hereditary inclusion body myositis associated with Paget's disease of the bone and frontotemporal dementia, many new mutations have been described in the last decade. " 10/01/2023 - "Arimoclomol is an oral co-inducer of the cellular heat shock response that was safe and well-tolerated in a pilot study of inclusion body myositis, reduced key pathological markers of inclusion body myositis in two in-vitro models representing degenerative and inflammatory components of this disease, and improved disease pathology and muscle function in mutant valosin-containing protein mice. "
5.	Acute Kidney Injury (Acute Renal Failure) 01/01/2022 - "In conclusion, these findings indicate that KUS121 can protect renal tubular cells from ER stress-induced injury, suggesting that KUS121 could be a novel and promising therapeutic compound for ischemia-associated AKI.NEW & NOTEWORTHY Novel findings of this study are as follows: 1) Kyoto University substance 121 (KUS121), a novel valosin-containing protein (VCP) modulator, can reduce ATP consumption of VCP; 2) KUS121 reduced endoplasmic reticulum (ER) stress and improved cell viability in proximal tubular cells; 3) KUS121 exerted renoprotective effects against ischemia-reperfusion injury; and 4) KUS121 may prevent ischemic acute kidney injury with ATP retention and restoring ER-associated degradation capacity." 01/01/2023 - ": AAA+: ATPases associated with diverse cellular activities; AKI: acute kidney injury; CBB: Coomassie Brilliant Blue; CRISPR: clustered regularly interspaced short palindromic repeats; ELDR: endo-lysosomal damage response; GFP: green fluorescent protein; GST: ‎glutathione S-transferase; IHC: immunohistochemistry; IP: immunoprecipitation; LAMP1: lysosomal-associated membrane protein 1; LC-MS: liquid chromatography-mass spectrometry; LGALS3/Gal3: galectin 3; LLOMe: L-leucyl-L-leucine methyl ester; MAP1LC3/LC3: microtubule associated protein 1 light chain 3; MEF: mouse embryonic fibroblast; PLAA: phospholipase A2, activating protein; PTP4A2: protein tyrosine phosphatase 4a2; PUB: NGLY1/PNGase/UBA- or UBX-containing protein; PUL: PLAP, Ufd3, and Lub1; TFEB: transcription factor EB; UBXN6/UBXD1: UBX domain protein 6; UPS: ubiquitin-proteasome system; VCP/p97: valosin containing protein; VCPIP1: valosin containing protein interacting protein 1; YOD1: YOD1 deubiquitinase." 01/01/2023 - ": AKI: acute kidney injury; AMBRA1: autophagy and beclin 1 regulator 1; ATP: adenosine triphosphate; BAK1: BCL2 antagonist/killer 1; BAX: BCL2 associated X, apoptosis regulator; BCL2: BCL2 apoptosis regulator; BECN1: beclin 1; BH3: BCL2 homology domain 3; BNIP3: BCL2 interacting protein 3; BNIP3L/NIX: BCL2 interacting protein 3 like; CASP1: caspase 1; CAT: catalase; CCCP: carbonyl cyanide m-chlorophenylhydrazone; CI-AKI: contrast-induced acute kidney injury; CISD1: CDGSH iron sulfur domain 1; CL: cardiolipin; CNP: 2',3'-cyclic nucleotide 3'-phosphodiesterase; DNM1L/DRP1: dynamin 1 like; E3: enzyme 3; ETC: electron transport chain; FA: folic acid; FUNDC1: FUN14 domain containing 1; G3P: glycerol-3-phosphate; G6PD: glucose-6-phosphate dehydrogenase; GPX: glutathione peroxidase; GSH: glutathione; GSK3B: glycogen synthase kinase 3 beta; GSR: glutathione-disulfide reductase; HIF1A: hypoxia inducible factor 1 subunit alpha; HUWE1: HECT, UBA and WWE domain containing 1; IL1B: interleukin 1 beta; IMM: inner mitochondrial membrane; IPC: ischemic preconditioning; IRI: ischemia-reperfusion injury; LIR: LC3-interacting region; LPS: lipopolysaccharide; MA: malate-aspartate; MPT: mitochondrial permeability transition; MUL1: mitochondrial E3 ubiquitin protein ligase 1; mtROS: mitochondrial ROS; NLR: NOD-like receptor; NLRP3: NLR family pyrin domain containing 3; NOX: NADPH oxidase; OGD-R: oxygen-glucose deprivation-reperfusion; OMM: outer mitochondrial membrane; OPA1: OPA1 mitochondrial dynamin like GTPase; OXPHOS: oxidative phosphorylation; PARL: presenilin associated rhomboid like; PINK1: PTEN induced kinase 1; PLSCR3: phospholipid scramblase 3; PMP: peptidase, mitochondrial processing; PRDX: peroxiredoxin; PRKN: parkin RBR E3 ubiquitin protein ligase; RPTC: rat proximal tubular cells; ROS: reactive oxygen species; SLC7A11/xCT: solute carrier family 7 member 11; SOD: superoxide dismutase; SOR: superoxide reductase; SQSTM1/p62: sequestosome 1; TCA: tricarboxylic acid; TIMM: translocase of inner mitochondrial membrane; TOMM: translocase of outer mitochondrial membrane; TXN: thioredoxin; VDAC: voltage dependent anion channel; VCP: valosin containing protein."

Related Drugs and Biologics

1.	Proteins (Proteins, Gene)
2.	arimoclomol
3.	Adenosine Triphosphate (ATP)
4.	KUS121
5.	Adenosine Triphosphatases (ATPase)
6.	Sirolimus (Rapamycin)
7.	Chloroquine (Aralen)
8.	Microtubule-Associated Proteins (Microtubule-Associated Protein 2)
9.	Progranulins
10.	Ubiquitin

Related Therapies and Procedures

1.	Radiotherapy
2.	Drug Therapy (Chemotherapy)
3.	Therapeutics
4.	Deep Brain Stimulation
5.	Castration