A tale of two tails: ciliary mechanotransduction in ADPKD

被引：13

作者：

Cantiello, HF ^{[1
]}

机构：

[1] Massachusetts Gen Hosp East, Renal Unit, Charlestown, MA 02129 USA

来源：

TRENDS IN MOLECULAR MEDICINE | 2003年 / 9卷 / 06期

关键词：

D O I：

10.1016/S1471-4914(03)00073-X

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Autosomal dominant polycystic kidney disease (ADPKD) is a common lethal genetic disorder, characterized by the progressive development of fluid-filled cysts in the kidney, pancreas and liver, and anomalies of the cardiovascular system. Mutations in PKD1 and PKD2, which encode the transmembrane proteins polycystin-1 (PC1) and polycystin-2 (PC2) respectively, account for almost all cases of ADPKD. However, the mechanisms by which abnormalities in PKD1 and PKD2 lead to aberrant kidney development remain unknown. Recent progress in the understanding of ADPKD has focused on primary cilia, which act as sensory transducers in renal epithelial cells. New evidence shows that a mechanosensitive signal, cilia bending, activates the PC1-PC2 channel complex. When working properly, this functional complex elicits a transient Ca2+ influx, which is coupled to the release of Ca2+ from intracellular stores.

引用

页码：234 / 236

页数：3

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