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charged multivesicular body protein 4B; chromatin-modifying protein 4B; CHMP4b; SNF7 homolog associated with Alix 1; SNF7-2; hSnf7-2; vacuolar protein sorting-associated protein 32-2; Vps32-2; hVps32-2 (CHMP4B, C20orf178, SHAX1)
Function:
- component of ESCRT3 in the multivesicular body pathway
- within ESCRT3, serves as an acceptor for ESCRT1 complex
- interacts with CHMP2A, CHMP4A, CHMP4C, CHMP6 within ESCRT3
- interacts with ESCRT1 component PDCD6IP
- when overexpressed, membrane-assembled circular arrays of CHMP4B filaments can promote or stabilize negative curvature & outward budding
- interacts with CHMP6, CHMP4C & CHMP7
- interacts with VPS4A & VPS4B
- interaction with misfolded CFTR
Structure:
- the acidic C-terminus & the basic N-termminus are thought to render the protein in a closed, soluble & inactive conformation through an autoinhibitory intramolecular interaction
- the open & active conformation, which enables membrane binding & oligomerization, is facilitated by interaction with other ESCRT components
- belongs to the SNF7 family
Compartment: cytoplasm, late endosome membrane
Expression:
- widely expressed
- expressed in heart, skeletal muscle > brain, colon, thymus, spleen, kidney, liver, small intestine, placenta, lung, peripheral blood lymphocytes.
Pathology:
- overexpression strongly inhibits HIV-1 release
- defects in CHMP4B are the cause of cataract posterior polar type 3
General
charged multivesicular body protein (CHMP)
Properties
SIZE: entity length = 224 aa
MW = 25 kD
COMPARTMENT: cytoplasm
MOTIF: acetylation site
SITE: N-TERMINUS
EFFECTOR-BOUND: acetyl
coiled coil {23-183}
Database Correlations
OMIM correlations
MORBIDMAP 610897
UniProt Q9H444
Pfam PF03357
Entrez Gene 128866
Kegg hsa:128866
References
UniProt :accession Q9H444
Component-of
endosomal sorting complex required for transport-3 (ESCRT3)