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1: Handb Exp Pharmacol. 2007;(179):363-75.Click here to read Links

TRPP2 channel regulation.

Witzgall R.

Institute for Molecular and Cellular Anatomy, University of Regensburg, Universitätsstrasse 31, 93053 Regensburg, Germany. ralph.witzgall@vkl.uni-regensburg.de

Polycystin-2, or TRPP2 according to the TRP nomenclature, is encoded by PKD2, a gene mutated in patients with autosomal-dominant polycystic kidney disease. Its precise subcellular location and its intracellular trafficking are a matter of intense debate, although consensus has emerged that it is located in primary cilia, a long-neglected organelle possibly involved in sensory functions. Polycystin-2 has a calculated molecular mass of 110 kDa, and according to structural predictions it contains six membrane-spanning domains and a pore-forming region between the 5th and 6th membrane-spanning domain. This section irst introduces the reader to the field of cystic kidney diseases and to the PKD2 gene, before the ion channel properties of polycystin-2 are discussed in great detail.

PMID: 17217069 [PubMed - indexed for MEDLINE]

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