Abstract

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 a 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 first 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.

Keywords

Polycystic kidney disease Cation channel Mechanosensation Chemosensation Primary cilia 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2007

Authors and Affiliations

  • R. Witzgall
    • 1
  1. 1.Institute for Molecular and Cellular AnatomyUniversity of RegensburgRegensburgGermany

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