Target intelligence / Profile preview

VPS33B-interacting protein, apical-basolateral polarity regulator, SPE-39 homolog (VIPAS39)

Target
VIPAS39
Molecular classification
Other (adaptor/scaffold protein, vesicle tethering complex component)
01

Overview

VIPAS39 (VPS33B-interacting protein, apical-basolateral polarity regulator, SPE-39 homolog) is a protein involved in vesicle trafficking, specifically regulating the transport of proteins from endosomes to lysosomes and maintaining epithelial cell polarity through its interaction with VPS33B and the HOPS complex. VIPAS39 localizes predominantly to the Golgi apparatus and endosomes and plays a role in the apical recycling pathway in epithelial cells. Loss-of-function mutations in VIPAS39 are associated with arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome, highlighting its crucial role in intracellular trafficking and organ development. It may also participate in collagen metabolic processes and downstream regulation of proteins critical for cell adhesion[1][4][9].

Other names
VPS16BSPE-39SPE39VIPARC14orf133hSPE-39VPS33B-interacting protein involved in polarity and apical protein restrictionVPS33B-interacting protein in apical-basolateral polarity regulatorVPS33B-interacting protein in polarity and apical restrictionChromosome 14 open reading frame 133
02

Biological functions

Endosome to lysosome transportIntracellular protein transportLysosomal traffickingApical-basolateral polarity maintenance in epithelial cellsCollagen metabolic processPeptidyl-lysine hydroxylationSpermatogenesisPossibly indirect transcriptional regulation of E-cadherin
03

Disease associations

Arthrogryposis, renal dysfunction, and cholestasis (ARC) syndromeOther congenital disorders of glycosylation and lysosomal storage

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