Recombinant Human Charged multivesicular body protein 2b (CHMP2B)

Uniprot Id

Q9UQN3

Target Species

Human

Target Name

CHMP2B

Target Full Name

Charged multivesicular body protein 2b

Target Function

Probable core component of the endosomal sorting required for transport complex III (ESCRT-III) which is involved in multivesicular bodies (MVBs) formation and sorting of endosomal cargo proteins into MVBs. MVBs contain intraluminal vesicles (ILVs) that are generated by invagination and scission from the limiting membrane of the endosome and mostly are delivered to lysosomes enabling degradation of membrane proteins, such as stimulated growth factor receptors, lysosomal enzymes and lipids. The MVB pathway appears to require the sequential function of ESCRT-O, -I,-II and -III complexes. ESCRT-III proteins mostly dissociate from the invaginating membrane before the ILV is released. The ESCRT machinery also functions in topologically equivalent membrane fission events, such as the terminal stages of cytokinesis and the budding of enveloped viruses (HIV-1 and other lentiviruses). ESCRT-III proteins are believed to mediate the necessary vesicle extrusion and/or membrane fission activities, possibly in conjunction with the AAA ATPase VPS4.

Target Involvement

Frontotemporal dementia, chromosome 3-linked (FTD3); Amyotrophic lateral sclerosis 17 (ALS17)

Target Subcellular Location

Cytoplasm, cytosol. Late endosome membrane; Peripheral membrane protein.

Target Protein Families

SNF7 family

Target Tissue Specificity

Widely expressed. Expressed in brain, heart, skeletal muscle, spleen, kidney, liver, small intestine, pancreas, lung, placenta and leukocytes. In brain, it is expressed in cerebellum, cerebral cortex, medulla, spinal chord, occipital lobe, frontal lobe, t

Target Research Area

Signal Transduction

Target Synonyms

ALS17; Charged multivesicular body protein 2b; CHM2B_HUMAN; CHMP family; member 2B; CHMP2.5; CHMP2b; Chromatin modifying protein 2b; Chromatin-modifying protein 2b; DMT1; hVps2-2; Vacuolar protein sorting 2; yeast; homolog of; B; Vacuolar protein sorting 2-2; Vacuolar protein sorting-associated protein 2-2; VPS2 homolog B; Vps2-2; VPS2B

Target Background

This gene encodes a component of the heteromeric ESCRT-III complex (Endosomal Sorting Complex Required for Transport III) that functions in the recycling or degradation of cell surface receptors. ESCRT-III functions in the concentration and invagination of ubiquitinated endosomal cargos into intralumenal vesicles. The protein encoded by this gene is found as a monomer in the cytosol or as an oligomer in ESCRT-III complexes on endosomal membranes. It is expressed in neurons of all major regions of the brain. Mutations in this gene result in one form of familial frontotemporal lobar degeneration.