IPR006228
Polycystin cation channel
InterPro entry
Short name | Polycystin_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
Contributing Member Database Entry
- NCBIfam:TIGR00864