| Primary Identifier | IPR052387 | Type | Family |
| Short Name | Fibrocystin |
| description | This family of proteins is involved in promoting ciliogenesis within renal epithelial cells, contributing to the formation of kidney tubules and maintaining the lumen architecture [, ]. They play a role in cellular symmetry, ensuring proper bipolar cell division by regulating centrosome duplication and mitotic spindle assembly []. These proteins also maintain oriented cell division during tubular elongation via the planar cell polarity pathway. They are involved in epithelial cell morphogenesis, regulating cell-cell and cell-matrix adhesion, and participating in cell motility. They enhance cell-cell contact by positively regulating PTK2 kinase activity, which can lead to increased epithelial cell proliferation through HRAS/RAF1 pathways or decreased apoptosis via the PDK1/AKT1 pathway []. Additionally, they may have roles in collecting-duct and biliary differentiation, as well as in the proliferation of cholangiocytes and the production of CCN2 in a CXCL8-dependent manner []. |
