background:
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. [provided by RefSeq, Jul 2008]
Function:
Necessary for the fragmentation of Golgi stacks during mitosis and for their reassembly after mitosis. Involved in the formation of the transitional endoplasmic reticulum (tER). The transfer of membranes from the endoplasmic reticulum to the Golgi apparatus occurs via 50-70 nm transition vesicles which derive from part-rough, part-smooth transitional elements of the endoplasmic reticulum (tER). Vesicle budding from the tER is an ATP-dependent process. The ternary complex containing UFD1L, VCP and NPLOC4 binds ubiquitinated proteins and 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 and is necessary for the formation of a closed nuclear envelope (By similarity). Regulates E3 ubiquitin-protein ligase activity of RNF19A.
Subcellular Location:
Cytoplasm > cytosol. Nucleus. Present in the neuronal hyaline inclusion bodies specifically found in motor neurons from amyotrophic lateral sclerosis patients. Present in the Lewy bodies specifically found in neurons from Parkinson disease patients.
Post-translational modifications:
Phosphorylated by tyrosine kinases in response to T-cell antigen receptor activation (By similarity).
Phosphorylated upon DNA damage, probably by ATM or ATR.
ISGylated.
DISEASE:
Defects in VCP are the cause of inclusion body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD) [MIM:16764]; also known as muscular dystrophy, limb-girdle, with Paget disease of bone or pagetoid amyotrophic lateral sclerosis or pagetoid neuroskeletal syndrome or lower motor neuron degeneration with Paget-like bone disease. IBMPFD features adult-onset proximal and distal muscle weakness (clinically resembling limb girdle muscular dystrophy), early-onset Paget disease of bone in most cases and premature frontotemporal dementia.
Similarity:
Belongs to the AAA ATPase family.
Database links:
Entrez Gene: 7415 Human
Entrez Gene: 269523 Mouse
Entrez Gene: 116643 Rat
Omim: 601023 Human
SwissProt: P55072 Human
SwissProt: Q01853 Mouse
SwissProt: P46462 Rat
Unigene: 529782 Human
Unigene: 245976 Mouse
Unigene: 98891 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
含缬酪肽蛋白(VCP即p97),是一种广泛存在的膜结合糖蛋白,在细胞活性中有着广泛的功能,其特点是作为类似分子伴侣的作用在内质网相关的蛋白降解及细胞周期调控中起到重要作用，VCP蛋白可以和肝素在细胞表面结合，而起到保护细胞的作用。
目前对VCP的表达水平与食道癌、胃癌、结、直肠癌、肝癌、胰腺癌等消化系统恶性肿瘤等是研究的热点。
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