Microtubular regulation of polycystin-2 in primary cilia 1 POLYCYSTIN-2 CATION CHANNEL FUNCTION IS UNDER THE CONTROL OF MICROTUBULAR STRUCTURES IN PRIMARY CILIA OF RENAL EPITHELIAL CELLS
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POLYCYSTIN-2 CATION CHANNEL FUNCTION IS UNDER THE CONTROL OF MICROTUBULAR STRUCTURES IN PRIMARY CILIA OF RENAL EPITHELIAL CELLS Qiang Li*, Nicolás Montalbetti*, Yuliang Wu, Arnolt Ramos, Malay K. Raychowdhury, Xing-Zhen Chen and Horacio F. Cantiello From the Department of Physiology, University of Alberta, Edmonton, T6G 2H7, Canada; the Laboratorio de Canales Iónicos, Departamento de Fisicoquímica y Química Analítica, Facultad de Farmacia y Bioquímica, Buenos Aires, 1113 Argentina; and the Renal Unit, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA 02129
منابع مشابه
Polycystin-2 cation channel function is under the control of microtubular structures in primary cilia of renal epithelial cells.
Mutations in the gene encoding polycystin-2 (PC2) result in autosomal dominant polycystic kidney disease and defects in left-right asymmetry during embryogenesis. PC2 is a TRP-type Ca(2+)-permeable non-selective cation channel, which is expressed in kidney and other organs. PC2 is present and functional in microtubule-containing primary cilia of renal epithelial cells. However, no information i...
متن کاملPolycystin-2 is an essential ion channel subunit in the primary cilium of the renal collecting duct epithelium
Mutations in the polycystin genes, PKD1 or PKD2, results in Autosomal Dominant Polycystic Kidney Disease (ADPKD). Although a genetic basis of ADPKD is established, we lack a clear understanding of polycystin proteins' functions as ion channels. This question remains unsolved largely because polycystins localize to the primary cilium - a tiny, antenna-like organelle. Using a new ADPKD mouse mode...
متن کاملORE CURRICULUM IN NEPHROLOGY Cystic and Inherited Kidney Diseases
● Mutations in the polycystic kidney disease (PKD)1 gene account for approximately 85% of affected families.3 ● The PKD1 gene is located on the short arm of chromosome 16 (16p.3.3). PKD1 codes for a 4,304-amino-acid protein (polycystin 1)2 with as yet undefined function but interacts with polycystin 2 and is involved in cell cycle regulation and intracellular calcium transport. Polycystin 1 loc...
متن کاملMore than colocalizing with polycystin-1, polycystin-L is in the centrosome.
Polycystin-1 and polycystin-2 are involved in autosomal dominant polycystic kidney disease by unknown mechanisms. These two proteins are located in primary cilia where they mediate mechanosensation, suggesting a link between cilia function and renal disease. In this study, we sought to characterize the subcellular localization of polycystin-L, a closely related member of polycystin-2, in epithe...
متن کاملPolycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease
Defects in the PKD1 and PKD2 genes cause autosomal dominant polycystic kidney disease (PKD) in ~1 in 1000 adults worldwide. These genes encode polycystin-1 and polycystin-2, which are membrane proteins thought to be involved in a calcium signal transduction cascade that controls epithelial proliferation and differentiation. Individuals with mutations in these genes develop cysts in the ducts an...
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