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valosin-containing protein; transitional endoplasmic reticulum ATPase; TER ATPase; 15S Mg+2-ATPase p97 subunit (VCP)
Function:
- necessary for fragmentation of Golgi stacks during mitosis & for their reassembly after mitosis
- involved in formation of the transitional ER (t-ER) (see endoplasmic reticulum)
- the ternary complex containing UFD1L, VCP & NPLOC4 binds ubiquitinated proteins & is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome
- the NPLOC4-UFD1L-VCP complex regulates spindle disassembly at the end of mitosis & is necessary for the formation of a closed nuclear envelope
- part of a ternary complex containing STX5A, NSFL1C & VCP
- NSFL1C forms a homotrimer that binds to one end of a VCP homohexamer
- the complex binds to membranes enriched in phosphatidylethanolamine-containing lipids
- interaction with VCIP135 leads to dissociation of the complex via ATP hydrolysis by VCP
- part of a ternary complex containing NPLOC4, UFD1L & VCP
- interacts with NSFL1C-like protein p37; the complex has membrane fusion activity & is required for Golgi & endoplasmic reticulum biogenesis
- interacts with SELS/VIMP & SYVN1, as well as with DERL1, DERL2 & DERL3; which probably transfer misfolded proteins from the ER to VCP
- interacts with SVIP
- component of a complex required to couple retrotranslocation, ubiquitination & deglycosylation composed of NGLY1, SAKS1, AMFR, VCP & RAD23B
- directly interacts with UBXD2 & RNF19A. interacts with CASR
- regulates E3 ubiquitin-protein ligase activity of RNF19A phosphorylated by tyrosine kinases in response to T-cell antigen receptor activation (putative)
- phosphorylated upon DNA damage, probably by ATM or ATR
Structure:
- homohexamer
- forms a ring-shaped particle of 12.5 nm diameter, that displays 6-fold radial symmetry
- belongs to the AAA ATPase family
Compartment:
- cytoplasm, nucleus
Pathology:
- present in the neuronal hyaline inclusion bodies specifically found in motor neurons from amyotrophic lateral sclerosis patients
- present in Lewy bodies specifically found in neurons from Parkinson disease patients
- defects in VCP are the cause of pagetoid ALS
General
adenosine triphosphatase (ATPase)
oligomerizing protein
phosphoprotein
Properties
SIZE: entity length = 806 aa
MW = 89 kD
COMPARTMENT: cytoplasm
cell nucleus
MOTIF: acetylation site
SITE: N-TERMINUS
EFFECTOR-BOUND: acetyl
Ser phosphorylation site {S7}
ATP-binding site
NAME: ATP-binding site
SITE: 245-252
ATP-binding site
NAME: ATP-binding site
SITE: 518-525
Ser phosphorylation site {S702}
Ser phosphorylation site {S705}
Ser phosphorylation site {S784}
Tyr phosphorylation site {Y805}
Database Correlations
OMIM 601023
MORBIDMAP 601023
UniProt P55072
PFAM correlations
Entrez Gene 7415
Kegg hsa:7415
References
- UniProt :accession P55072
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=VCP
Component-of
NGLY1-SAKS1-AMFR-VCP-RAD23B complex