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An important potential relationship between p97/VCP and autophagic proteins in preeclampsia.
This study identified two novel and one reported mutation in the VCP gene in patients with Frontotemporal Dementia, and provided neuropathological features of the two novel mutations (p.Thr262Ser and p.Arg159Ser) carriers.
A heterozygous VCP that segregated in an autosomal-dominant pattern was identified by genetic analysis
Our results identify how VCP is specifically targeted to ubiquitylated substrates in the BAG6 triage pathway and suggest that the degradation of ubiquitylated clients by the proteasome is reliant on the association of UBXN1 with ubiquitylated substrates and the catalytic activity of VCP.
Study describes that VCP has potentially strong disaggregase activity that is used to dissociate monomers from complex protein aggregates, such as polyglutamine-expanded Huntingtin-exon1 aggregates, leading to their intracellular clearance. Its N-terminal (Cdc48_N domain) interacts with the N-terminal 17-amino acid region of Huntingtin-exon1.
Mechanistically, parkin/PINK1 catalyze a rapid burst of Mfn2 phosphoubiquitination to trigger p97-dependent disassembly of Mfn2 complexes from the outer mitochondrial membrane, dissociating mitochondria from the endoplasmic reticulum.
An interconnected role of VCP/p97 and GCN2 in maintaining cancer cell metabolic and protein homoeostasis.
p97-mediated degradation, together with a reduction in CReP synthesis, is essential for timely stress-induced reduction of CReP levels and, consequently, for robust eIF2alpha phosphorylation to enforce the stress response.
a genetic analysis was performed which revealed a heterozygous mutation of the VCP gene, thus confirming the diagnosis of IBMPFD (I: nclusion B: ody M: yopathie with P: aget Disease of the Bones and F: ronto-temporal D: ementia).
study highlights the remarkable adaptability of p97 ATPase domain communication that enables escape from mechanistically distinct classes of cytotoxic p97 inhibitors.
We demonstrate that VCP regulates many nuclear and chromatin-associated proteins and promotes the degradation and activity of the transcription factor c-Myc.
The heterozygous R155C VCP mutation is toxic in humans and harmless in mice because of a low lever expression.
These results indicate that the outer membrane protein MCL1 is degraded by the VCP-UBXD1 complex and that the process is promoted by the presence of mutant Huntingtin.
Study results suggest that VCP recruitment to mitochondria by mtHtt is a crucial step in the initiation of neuropathology in Huntington's disease.
ASPL efficiently promotes p97 hexamer disassembly, resulting in the formation of stable p97:ASPL. Overproduction of ASPL disrupts p97 hexamer function in endoplasmic reticulum-associated protein degradation.
Data indicate that approximately 9% of patients with valosin-containing protein (VCP) mutations had an amyotrophic lateral sclerosis (ALS) phenotype, 4% had been diagnosed with Parkinson's disease (PD), and 2% had been diagnosed with Alzheimer's disease (AD).
CB-5083 decreases viability in multiple myeloma cell lines and patient-derived multiple myeloma cells, including those with background proteasome inhibitor (PI) resistance. CB-5083 has a unique mechanism of action that combines well with PIs, which is likely owing to the p97-dependent retro-translocation of the transcription factor, Nrf1, which transcribes proteasome subunit genes following exposure to a PI
a p97 mutant that causes inclusion body myopathy, Paget's disease of bone, and frontotemporal dementia unfolds substrate faster, suggesting that excess activity may underlie pathogenesis
Results report that VCP/p97 promotes the degradation of ubiquitylated GS, resulting in its accumulation in cells with compromised p97 function. Notably, p97 is also required for the degradation of all four known CRBN neo-substrates [IKZF1, IKZF3, CK1alpha, and GSPT1] whose ubiquitylation is induced by immunomodulatory drugs.
non-cell-autonomous effects of VCP-mutant astrocytes on both control and mutant Motor neurons, were examined.
VCP removes sterically trapped Ku70/80 rings from DNA in double-strand break repair.
Findings indicate that p97 plays a conserved role in dismantling the CMG helicase complex during different cellular events, but that distinct regulatory signals ultimately control when and where unloading takes place.
p97 is an essential regulator of DNA damage-dependent CDT1 destruction
CDC-48/p97 coordinates CDT-1 degradation with GINS chromatin dissociation to ensure faithful DNA replication
data reveal an essential pathway that regulates reformation of the nucleus after mitosis and defines ubiquitin-dependent protein extraction as a common mechanism of Cdc48/p97 activity also during nucleus formation
When we introduced CDC48 antisense morpholino oligonucleotides into zebrafish embryos, the morphant embryos were lethal and showed defects in neuronal outgrowth and neurodegeneration.
CDC48 may promote cell cycling and cell proliferation via C-terminal tyrosine phosphorylation during cold acclimation in fish cells
Both the mRNA and protein levels of Valosin-Containing Protein were regulated by Bap31 in vivo and in vitro.
The valosin-containing protein is a novel mediator of mitochondrial respiration and cell survival in the heart in vivo
Study reports for the first time that VCP is aberrantly translocated to the mitochondria and bound to mutant Huntingtin in a variety of Huntington's disease models.
Valosin-containing protein is a novel repressor of cardiomyocyte hypertrophy induced by pressure overload.
Together, these results suggested that mouse mammary tumor virus Rem uses a novel p97-dependent, Derlin-independent retrotranslocation mechanism distinct from other pathogens to avoid signal peptide ubiquitylation and proteasomal degradation.
cellular phenotypes caused by P137L mutant expression were not isolated observations, and some other IBMPFD disease-related VCP/p97 mutations could lead to similar outcomes
The functional motions of p97 using symmetric normal modes have been predicted.
results suggest that (i) NLRP3 inflammasome and local IL-1beta((+))F4/80((+))Ly6C((+)) inflammatory macrophages contribute to pathogenesis of VCP-associated myopathy
VCP (valosin-containing protein), together with its cofactor P47 and the endoplasmic reticulum (ER) morphology regulator ATL1 (Atlastin-1), regulates tubular ER formation
Lysine Methylation of the Valosin-Containing Protein (VCP) Is Dispensable for Development and Survival of Mice
Global expression profiling of VCP(R155H/+) mice identified key dysregulated signaling pathways including genes involved in the physiological system development and function, diseases and disorders, and molecular and cellular functions.
The results reveal an unexpected, crucial role of ATP consumption by VCP in determining cell fate in retinitis pigmentosa, and point to a promising new neuroprotective strategy for this currently incurable disease.
Pro(178) and Pro(183) of SelS play important roles in the translocation of p97(VCP) to the ER membrane and protect cells from ER stress
Knockdown or chemical inhibition of p97 causes robust accumulation of USP33 due to inhibition of its degradation
conclude that the Akt substrate, VCP, mediates the increased expression of iNOS downstream from Hsp22 through an NF-kappaB-dependent mechanism
Molecular dynamics simulations are used to probe energetic requirements of substrate protein translocation through the p97 pore and mechanisms of substrate binding and release from p97 subunits during the allosteric cycle.
Data indicate that genetic ablation or chemical inhibition of p97 does not diminish DRiP antigen presentation nor does it alter the levels of MHC class I molecules on the cell surface.
Cav-1 may be a cofactor in the interaction of Derlin-1 and N-glycosylated COX-2 and may facilitate Derlin-1- and p97 complex-mediated COX-2 ubiquitination, retrotranslocation, and degradation.
results suggest that VCP plays a mechanistic role in releasing WRNp from the nucleolus
rescue experiments with a lethal mfap1 mutant show that the VCP binding region is not essential for Mfap1 function, but may act to increase its stability or activity
Clu is upstream of and binds to VCP in vivo and promotes VCP-dependent Marf degradation in vitro Marf accumulates in whole muscle lysates of clu-deficient flies and is destabilized upon Clu overexpression. Thus, Clu is essential for mitochondrial homeostasis and functions in concert with Parkin and VCP for Marf degradation to promote damaged mitochondrial clearance.
results have revealed SUMOylation as a molecular signaling switch to regulate the distribution and functions of VCP during stress response, and suggest that deficiency in VCP SUMOylation caused by pathogenic mutations will render cells vulnerable to stress insults.
The authors propose that VCP sustains sarcoplasmic proteostasis, in part, by controlling the integrity of a dynamic tubular lysosomal network.
Ter94/VCP appears to be an evolutionarily conserved component that regulates BMP-Smad1/5/8 signaling.
Data identifies that ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS
Valosin-Containing Protein has an essential role in dendrite pruning through regulating mRNA metabolism
VCP was validated as an atlastin-interacting protein.
Ter94 ATPase and K11-linked ubiquitination in Ci contribute to the selectivity by proteasomes for partial degradation.
VCP binds to DIAP1 in a ubiquitin- and BIR domain-dependent manner and facilitates its degradation, therefore, establishing a new link between ubiquitin, dendrite pruning and the apoptosis machinery.
Subcellular localization and ATPase activity of VCP clearly influenced the localization of the aggregates throughout the cells.
Genetic inactivation of ter94 increased the levels of misfolded Rh1 in retina.
The protein encoded by this gene is a member of a family that includes putative ATP-binding proteins involved in vesicle transport and fusion, 26S proteasome function, and assembly of peroxisomes. This protein, as a structural protein, is associated with clathrin, and heat-shock protein Hsc70, to form a complex. It has been implicated in a number of cellular events that are regulated during mitosis, including homotypic membrane fusion, spindle pole body function, and ubiquitin-dependent protein degradation.
15S Mg(2+)-ATPase p97 subunit
, TER ATPase
, transitional endoplasmic reticulum ATPase
, valosin-containing protein
, yeast Cdc48p homolog
, p97 ATPase
, AAA ATPase p97
, Inv protein
, homolog of yeast cdc48
, valosin containing protein
, CG2331 gene product from transcript CG2331-RA
, TRANSITIONAL ENDOPLASMIC RETICULUM ATPASE TER94
, Valosin-Containing protein
, Valosin-containing protein homolog
, complementation group I
, transitional endoplasmic reticulum 94
, Fanconi anemia group G protein
, Fanconi anemia, complementation group G