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Vacuolar protein sorting-associated protein 51 subunit of GARP complex (VPS51)

Target
VPS51
Molecular classification
Multisubunit tethering complex subunit, Other (not a receptor, transporter, enzyme, ion channel, transcription factor, nor involved in histone modification)
01

Overview

VPS51 (Vacuolar protein sorting-associated protein 51 subunit of GARP complex) is one of four essential components of the Golgi-associated retrograde protein (GARP) complex, which also includes VPS52, VPS53, and VPS54[1][2][3]. The complex is located at the trans-Golgi network (TGN) and functions primarily to tether and facilitate the fusion of retrograde transport vesicles derived from endosomes to the TGN[1][3][4]. VPS51 is specifically required for the complex's association with SNARE proteins (notably the t-SNARE Tlg1p in yeast, orthologous to syntaxin 6 in mammals), regulating the retrieval of cargo and protein recycling from endosomes to the Golgi[2]. Deficiency or malfunction of VPS51 leads to defects in vesicle trafficking and lysosomal function, with disease links to pontocerebellar hypoplasia and lissencephaly[4]. VPS51’s molecular role is best classified as a vesicle tethering factor, with no direct evidence of receptor, enzyme, or transporter activity. It is not a current therapeutic drug target.

Other names
ANG2ANG3C11orf2C11orf3FFRPP5382PCH13Protein fat-free homologAnother new gene 2 proteinfat-free homolog (zebrafish)GARP complex subunit
02

Biological functions

Vesicle tethering in retrograde transportMaintenance of protein retrieval from endosomes to the trans-Golgi network (TGN)Sorting of acid hydrolasesLysosome functionGlycoprotein modification in the Golgi complex
03

Disease associations

Pontocerebellar hypoplasia, type 13LissencephalyOther (General defects in glycoprotein modification and trafficking may contribute to disease)

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