Vacuolar protein sorting-associated protein: Difference between revisions
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== Function == | == Function == | ||
'''Vacuolar protein sorting-associated protein''' (Vps) are part of '''E'''ndosomal '''S'''orting '''C'''omplex '''R'''equired for '''T'''ransport ('''ESCRT''') that performs the topologically unique membrane bending and scission reaction away from the cytoplasm. This process is required for the multivesicular body (MVP) pathway, cytokinesis and HIV budding. There are five distinct ESCRT complexes (0,I,II,III,Vps4) with distinct functions<ref>PMID:22361144</ref>. | '''Vacuolar protein sorting-associated protein''' (Vps) are part of '''E'''ndosomal '''S'''orting '''C'''omplex '''R'''equired for '''T'''ransport ('''ESCRT''') that performs the topologically unique membrane bending and scission reaction away from the cytoplasm. This process is required for the multivesicular body (MVP) pathway, cytokinesis and HIV budding. There are five distinct ESCRT complexes (0,I,II,III,Vps4) with distinct functions<ref>PMID:22361144</ref>. The '''retromer''' comprises 2 functional subcomplexes: the cargo-selective one which contains Vps35, Vps29 and Vps26 and the sorting nexin subcomplex which tubulate the endosomal membrane<ref>PMID:19531583</ref>. | ||
*'''Vps4''', '''Vps4A''', ''Vps4B'' complex catalyzes the disassembly of the ESCRT-III filament in an ATP-driven reaction. Vps4 proteins are required for centrosome and spindle maintenance<ref>PMID:20616062</ref>.<br /> | *'''Vps4''', '''Vps4A''', ''Vps4B'' complex catalyzes the disassembly of the ESCRT-III filament in an ATP-driven reaction. Vps4 proteins are required for centrosome and spindle maintenance<ref>PMID:20616062</ref>.<br /> | ||
*'''Vps9A''' activates Rab5 to GTP-bound form<ref>PMID:18055610</ref>.<br /> | *'''Vps9A''' activates Rab5 to GTP-bound form<ref>PMID:18055610</ref>.<br /> | ||
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== Disease == | == Disease == | ||
Mutations in Vps53 cause progressive cerebello-cerebral atrophy type 2<ref>PMID:24577744</ref>. Mutations in Vps54 in mice cause motor neuron disease<ref>PMID:16244655</ref>. | Mutations in '''Vps53''' cause progressive cerebello-cerebral atrophy type 2<ref>PMID:24577744</ref>. Mutations in '''Vps54''' in mice cause motor neuron disease<ref>PMID:16244655</ref>. Dysfunction of the '''retromer''' is implicated in neurodegenerative disease like Alzheimer disease, Parkinson's disease and Frontotemporal lobar degeneration<ref>PMID:29755290</ref>. | ||
== Relevance == | == Relevance == | ||