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Pflügers Archiv

, Volume 451, Issue 1, pp 264–276 | Cite as

Polycystins: polymodal receptor/ion-channel cellular sensors

  • Patrick Delmas
Invited Review

Abstract

Transient receptor potential (TRP) channel proteins are divided into seven subgroups that are currently designated as TRPC (canonical), TRPV (vanilloid), TRPM (melastatin), TRPN (NOMP-C, from no mechanoreceptor potential-C), TRPA (ankyrin-like with transmembrane domains 1) and TRPP (polycystin). TRPC, TRPV and TRPM are related to canonical TRP proteins whereas TRPN, TRPA and TRPP (polycystin) are more divergent. Most TRP channels are linked to sensory stimuli, including phototransduction, thermosensation and mechanosensation. The TRPP subfamily was named after its founding member, polycystin kidney disease-2 (PKD2), a gene product mutated in many cases of autosomal dominant polycystic kidney disease (ADPKD). ADPKD is a major inherited nephropathy, affecting over 1:1,000 of the worldwide population, characterized by the progressive development of fluid-filled cysts from the tubules and collecting ducts of affected kidneys. Loss-of-function mutations in either polycystin-2, a non-selective cation channel, or polycystin-1 (PKD1), a large plasma membrane integral protein, give rise to ADPKD. PKD1 and PKD2 are thought to function together as part of a multiprotein receptor/ion-channel complex or independently and may be involved in transducing Ca2+-dependent mechanosensitive signals in response to cilia bending in renal epithelial cells and endodermally derived cells. Further information on the growing number and physiological properties of these TRP-polycystins is the basis of this review.

Keywords

Polycystic kidney disease PKD1 PKD2 Polycystins TRP channels Mechanosensation Primary cilia Sensory transduction 

Notes

Acknowledgement

This work was supported by the Centre National de la Recherche Scientifique (CNRS).

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© Springer-Verlag 2005

Authors and Affiliations

  1. 1.Faculté de Médecine, IFR Jean Roche, Laboratoire de Neurophysiologie CellulaireCNRS-UMR 6150Marseille Cedex 20France

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