Uroplakin-3a (IPR024825)

Short name: Uroplakin-3a

Overlapping homologous superfamilies


Family relationships


This entry represents uroplakin-3a. Defects in uroplakin-3a are a cause of renal adysplasia, a condition in which one or both kidneys are absent at birth [PMID: 15888565, PMID: 16731295].

Uroplakins (UPs) are a family of proteins that associate with each other to form plaques on the apical surface of the urothelium, the pseudo-stratified epithelium lining the urinary tract from renal pelvis to the bladder outlet [PMID: 27729990, PMID: 17183244]. UPs are classified into 3 types: UPIa and UPIb, UPII, and UPIIIa and IIIb. UPIs are tetraspanins that have four transmembrane domains separating one large and one small extracellular domain while UPII and UPIIIs are single-pass transmembrane proteins. UPIa and UPIb form specific heterodimers with UPII and UPIII, respectively, which allows them to exit the endoplasmatic rediculum. UPII/UPIa and UPIIIs/UPIb form heterotetramers and six of these tetramers form the 16nm particle, seen in the hexagonal array of the asymmetric unit membrane, which is believed to form a urinary tract barrier [PMID: 7760330]. Uroplakins are also believed to play a role during urinary tract morphogenesis [PMID: 19340092, PMID: 17183244].

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.