Valosin Containing Protein

From Anvita Health Wiki

Contents 1 Function 2 Structure 3 Compartment 4 Pathology 5 More General Terms 6 Internet Database 7 References

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

More General Terms

• adenosine triphosphatase (ATPase)
• oligomerizing protein
• phosphoprotein

Internet Database

Kegg: [1]
OMIM: 601023
Prosite: [2]
UniProt: [3]
Entrez gene: 7415

References

1. UniProt [4]
2. GeneReviews [5]

valosin-containing protein; transitional endoplasmic reticulum ATPase; TER ATPase; 15S Mg+2-ATPase p97 subunit (VCP)