Polycystin-2 is an intracellular calcium release channel View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2002-02-18

AUTHORS

Peter Koulen, Yiqiang Cai, Lin Geng, Yoshiko Maeda, Sayoko Nishimura, Ralph Witzgall, Barbara E. Ehrlich, Stefan Somlo

ABSTRACT

Polycystin-2, the product of the gene mutated in type 2 autosomal dominant polycystic kidney disease (ADPKD), is the prototypical member of a subfamily of the transient receptor potential (TRP) channel superfamily, which is expressed abundantly in the endoplasmic reticulum (ER) membrane. Here, we show by single channel studies that polycystin-2 behaves as a calcium-activated, high conductance ER channel that is permeable to divalent cations. Epithelial cells overexpressing polycystin-2 show markedly augmented intracellular calcium release signals that are lost after carboxy-terminal truncation or by the introduction of a disease-causing missense mutation. These data suggest that polycystin-2 functions as a calcium-activated intracellular calcium release channel in vivo and that polycystic kidney disease results from the loss of a regulated intracellular calcium release signalling mechanism. More... »

PAGES

191-197

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/ncb754

DOI

http://dx.doi.org/10.1038/ncb754

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1042398135

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/11854751


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