VCP(Valosin-containing protein) belongs to the AAA ATPase family.VCP was first identified as a result of attempts to clone a putative peptide hormone called valosin. It was found that the cloned cDNA encoded a ubiquitously expressed 90 kDa cytosolic protein,termed VCP,which showed none of the characteristics of a peptide hormone precursor.Defects in VCP are the cause of inclusion body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD) and amyotrophic lateral sclerosis type 14 with or without frontotemporal dementia (ALS14)
A synthesized peptide derived from human VCY; VCY1B.
A synthesized peptide derived from human VDAC1, corresponding to a region within C-terminal amino acids.
Non-selective voltage-gated ion channel that mediates the transport of anions and cations through the mitochondrion outer membrane and plasma membrane. The channel at the outer mitochondrial membrane allows diffusion of small hydrophilic molecules; in the plasma membrane it is involved in cell volume regulation and apoptosis. It adopts an open conformation at low or zero membrane potential and a closed conformation at potentials above 30-40 mV. The open state has a weak anion selectivity whereas the closed state is cation-selective. Binds various signaling molecules, including the sphingolipid ceramide, the phospholipid phosphatidylcholine, and the sterols cholesterol and oxysterol. In depolarized mitochondria, acts downstream of PRKN and PINK1 to promote mitophagy or prevent apoptosis; polyubiquitination by PRKN promotes mitophagy, while monoubiquitination by PRKN decreases mitochondrial calcium influx which ultimately inhibits apoptosis. May participate in the formation of the permeability transition pore complex (PTPC) responsible for the release of mitochondrial products that triggers apoptosis. May mediate ATP export from cells. Part of a complex composed of HSPA9, ITPR1 and VDAC1 that regulates mitochondrial calcium-dependent apoptosis by facilitating calcium transport from the ER lumen to the mitochondria intermembrane space thus providing calcium for the downstream calcium channel MCU that directly releases it into mitochondria matrix. Mediates cytochrome c efflux. Catalyzes the scrambling of phospholipids across the outer mitochondrial membrane; the mechanism is unrelated to channel activity and is capable of translocating both anionic and zwitterionic phospholipids.
This gene encodes a voltage-dependent anion channel protein that is a major component of the outer mitochondrial membrane. The encoded protein facilitates the exchange of metabolites and ions across the outer mitochondrial membrane and may regulate mitochondrial functions. This protein also forms channels in the plasma membrane and may be involved in transmembrane electron transport. Alternate splicing results in multiple transcript variants. Multiple pseudogenes of this gene are found on chromosomes 1, 2 3, 6, 9, 12, X and Y.
VDAC1,also named as VDAC,Porin 31HM,Porin 31HL and Plasmalemmal porin,belongs to the eukaryotic mitochondrial porin family. It adopts an open conformation at low or zero membrane potential and a closed conformation at potentials above 30-40 mV,to form a channel through the mitochondrial outer membrane and also the plasma membrane. Unlike other membrane transport proteins,porins are large enough to allow passive diffusion.
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