New Insights into the Molecular Mechanisms Targeting Tubular Channels/Transporters in PKD Development

Overview

Journal Kidney Dis (Basel)

Specialty Nephrology

Date 2016 Dec 7

PMID 27921040

Citations 5

Authors

Ming Wu

Shengqiang Yu

Affiliations

Soon will be listed here.

Abstract

Background: Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in the , or gene, which encodes polycystin-1, polycystin-2 or fibrocystin, respectively. Embryonic and postnatal mutation studies show that transport or channel function is dysregulated before the initiation of cystogenesis, suggesting that the abnormality of transport or channel function plays a critical role in the pathology of PKD.

Summary: Polycystin-2 by itself is a calcium-permeable cation channel, and its channel function can be regulated by polycystin-1 or fibrocystin. In this paper, we reviewed the current knowledge about calcium transports and cyclic adenosine monophosphate (cAMP)-driven chloride transports in PKD. In addition, the function and the underlining mechanism of glucose transporters, phosphate transporters and water channels in PKD are also discussed.

Key Messages: Abnormalities in calcium handling and exuberant cAMP-dependent cystic fibrosis transmembrane conductance regulator-mediated fluid secretion in the collecting duct are the most important issues in the pathogenesis of PKD.

Citing Articles

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Recent advances in understanding ion transport mechanisms in polycystic kidney disease.

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Prenatal genetic considerations of congenital anomalies of the kidney and urinary tract (CAKUT).

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Szabo T, Orosz P, Balogh E, Javorszky E, Mattyus I, Bereczki C Pediatr Nephrol. 2018; 33(10):1713-1721.

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