Fibrocystin


Fibrocystin is a large, receptor-like protein that is thought to be involved in the tubulogenesis and/or maintenance of duct-lumen architecture of epithelium.
FPC associates with the primary cilia of epithelial cells and co-localizes with the Pkd2 gene product polycystin-2, suggesting that these two proteins may function in a common molecular pathway.

Pathology

Mutations of its encoding gene can cause autosomal recessive polycystic kidney disease.