D
IPR006228

Polycystin cation channel

InterPro entry
Short namePolycystin_cat

Description

This represents the N-terminal region of polycystin, a member of the Polycystin Cation Channel (PCC) family. Polycystin is a huge protein of 4303aa. Its repeated leucine-rich (LRR) segment is found in many proteins. It contains 16 polycystic kidney disease (PKD) domains, one LDL-receptor class A domain, one C-type lectin family domain, and 16-18 putative transmembrane regions in positions between residues 2200 and 4100. Polycystin-L has been shown to be a cation (Na+, K+and Ca2+) channel that is activated by Ca2+. Two members of the PCC family (polycystin 1 and 2) are mutated in autosomal dominant polycystic kidney disease, and polycystin-L is deleted in mice with renal and retinal defects
[1, 2]
.

References

1.The structure of a PKD domain from polycystin-1: implications for polycystic kidney disease. Bycroft M, Bateman A, Clarke J, Hamill SJ, Sandford R, Thomas RL, Chothia C. EMBO J. 18, 297-305, (1999). View articlePMID: 9889186

2.Polycystin-L is a calcium-regulated cation channel permeable to calcium ions. Chen XZ, Vassilev PM, Basora N, Peng JB, Nomura H, Segal Y, Brown EM, Reeders ST, Hediger MA, Zhou J. Nature 401, 383-6, (1999). View articlePMID: 10517637

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