Abstract | PURPOSE OF REVIEW: RECENT FINDINGS: The precise functions of polycystin-1 and polycystin-2 are unclear. However, recent work has revealed that polycystin-1 may induce or modulate ion channels, including polycystin-2 channels, and that polycystin-2 functions as a calcium-regulated, calcium-permeable cation channel on the endoplasmic reticulum or on the plasma membrane with polycystin-1. These data suggest that ion-channel signaling mediated by polycystins is important for tubule formation in kidney and that disrupted signaling results in cyst formation. SUMMARY:
ADPKD is a systemic hereditary disease that is characterized by renal and hepatic cysts, and results in end-stage renal failure in 50% of affected individuals. Most cases (>95%) are caused by genetic mutations in either the PKD1 or the PKD2 gene, or both, which encode polycystin-1 and polycystin-2, respectively. The present review provides a hint of how malfunction of polycystins may give rise to cysts, based on recent observations concerning polycystin channels. Polycystin channel signaling may prove to be an important new target for therapy of ADPKD.
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Authors | Masahiro Ikeda, William B Guggino |
Journal | Current opinion in nephrology and hypertension
(Curr Opin Nephrol Hypertens)
Vol. 11
Issue 5
Pg. 539-45
(Sep 2002)
ISSN: 1062-4821 [Print] England |
PMID | 12187319
(Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S., Review)
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Chemical References |
- Cations
- Ion Channels
- Membrane Proteins
- Proteins
- TRPP Cation Channels
- polycystic kidney disease 1 protein
- polycystic kidney disease 2 protein
- Protein Kinase C
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Topics |
- Calcium Signaling
- Cations
(metabolism)
- Humans
- Ion Channels
(metabolism)
- Membrane Proteins
(chemistry, metabolism)
- Protein Kinase C
(metabolism)
- Protein Structure, Tertiary
- Proteins
(chemistry, metabolism)
- Signal Transduction
- TRPP Cation Channels
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