Fibrocystin

Definition:

Promotes ciliogenesis in renal epithelial cells and therefore participates in the tubules formation and/ or ensures the maintenance of the architecture of the lumen of the kidney (By similarity). Has an impact on cellular symmetry by ensuring correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly and by maintaining oriented cell division (OCD) during tubular elongation through planar cell polarity (PCP) pathway. During epithelial cell morphogenesis regulates also cell-cell and cell-matrix adhesion and participates in cell motility (By similarity). Promotes cell-cell contact through the positive regulation of PTK2 kinase activity leading to either positive regulation of epithelial cell proliferation through the HRAS/RAF1 pathways, or negative regulation of apoptosis through the PDK1/AKT1 pathway (By similarity). May act in collecting-duct and biliary differentiation. May participate in the regulation of the cholangiocytes proliferation and the CCN2 production in an CXCL8-dependent manner.

References:

[1]. Christopher J Ward, et al. The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet. 2002 Mar;30(3):259-69. [Content Brief]

[2]. Tomoyuki Tsunoda, et al. Loss of fibrocystin promotes interleukin-8-dependent proliferation and CTGF production of biliary epithelium. J Hepatol. 2019 Jul;71(1):143-152. [Content Brief]

[3]. Jingjing Zhang, et al. Polycystic kidney disease protein fibrocystin localizes to the mitotic spindle and regulates spindle bipolarity. Hum Mol Genet. 2010 Sep 1;19(17):3306-19. [Content Brief]

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