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Rabbit Anti-UPK3A/FITC Conjugated antibody
background:
The asymmetric unit membrane (AUM) forms numerous plaques, which cover the apical surface of the urothelium. These plaques are thought to strengthen the urothelium and reduce the risk of rupturing during ladder distention. They are composed of four major integral membrane proteins called uroplakins (UP). The uroplakin family comprises UPIa, UPIb, UPII, and UPIII. Family members are conserved among several species, including human, mouse, rat, rabbit, dog, pig and sheep. UPIa and UPIb form tightly packed structures with UPII and UPIII, respectively. This pairing is required for normal urothelial plaque formation and is regulated by proteolytic processing of the uroplakin proteins. Uroplakins are expressed in normal urothelium and are used as specific markers of urothelial differentiation. They are also expressed in a majority of transitional cell carcinomas of the bladder (TCCs), which make the uroplakins a useful marker for detecting bladder cancer metastasis and for staging and monitoring chemotherapeutic response.
Function:
Component of the asymmetric unit membrane (AUM); a highly specialized biomembrane elaborated by terminally differentiated urothelial cells. May play an important role in AUM-cytoskeleton interaction in terminally differentiated urothelial cells. It also contributes to the formation of urothelial glycocalyx which may play an important role in preventing bacterial adherence.
Subcellular Location:
Endoplasmic reticulum membrane. Heterodimer formation with UPK1B is a prerequisite to exit out of the endoplasmic reticulum (ER).
Tissue Specificity:
Expressed in ureter.
DISEASE:
Defects in UPK3A are a cause of renal adysplasia (RADYS) [MIM:191830]; also known as renal agenesis or renal aplasia. Renal agenesis refers to the absence of one (unilateral) or both (bilateral) kidneys at birth. Bilateral renal agenesis belongs to a group of perinatally lethal renal diseases, including severe bilateral renal dysplasia, unilateral renal agenesis with contralateral dysplasia and severe obstructive uropathy.
Similarity:
Belongs to the uroplakin-3 family.
Database links:
Entrez Gene: 7380 Human
Omim: 611559 Human
SwissProt: O75631 Human
SwissProt: Q32N05 Human
Unigene: 632787 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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