Deficiency of polycystin-2 reduces Ca super(2+) channel activity and cell proliferation in ADPKD lymphoblastoid cells

Polycystin-2 (PC2), the product of the PKD2 gene mutated in type 2 autosomal-dominant polycystic kidney disease (ADPKD), is a Ca super(2+)-permeable cation channel found in kidney epithelia and other tissues. The precise expression and subcellular location of PC2 are controversial and there is littl...

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Published in:The FASEB journal 2004-05, Vol.18 (7), p.884-886
Main Authors: Aguiari, Gianluca, Banzi, Manuela, Gessi, Stefania, Cai, Yiqiang, Zeggio, Emanuela, Manzati, Elisa, Piva, Roberta, Lambertini, Elisabetta, Ferrari, Luisa, Peters, Dorien J, Lanza, Francesco, Harris, Peter C, Borea, Pier Andrea, Somlo, Stefan, Del Senno, Laura
Format: Article
Language:English
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Summary:Polycystin-2 (PC2), the product of the PKD2 gene mutated in type 2 autosomal-dominant polycystic kidney disease (ADPKD), is a Ca super(2+)-permeable cation channel found in kidney epithelia and other tissues. The precise expression and subcellular location of PC2 are controversial and there is little information on the role of the endogenous protein in non-epithelial cells. Since PC2 is also expressed in B-lymphoblastoid cell lines (LCLs), where Ca super(2+) signaling pathways are important regulators of functional activities, we investigated whether PC2 might play some function in B-LCLs, taking advantage of the reduced levels of PC2 in LCLs from PKD2 subjects.
ISSN:0892-6638
DOI:10.1096/fj.03-0687fje