Ciliary Ion Channels in Polycystic Kidney Disease DOI Creative Commons
Lubna A. Alshriem,

Raghad Buqaileh,

Qasim Alorjani

et al.

Cells, Journal Year: 2025, Volume and Issue: 14(6), P. 459 - 459

Published: March 19, 2025

Polycystic kidney disease (PKD) is the most common hereditary disorder that disrupts renal function and frequently progresses to end-stage disease. Recent advances have elucidated critical role of primary cilia ciliary ion channels, including transient receptor potential (TRP) cystic fibrosis transmembrane conductance regulator (CFTR), polycystin in pathogenesis PKD. While some channels primarily as chloride (e.g., CFTR), others regulate calcium (Ca+2) homeostasis. These are essential for cellular signaling maintaining normal architecture. Dysregulation these pathways due genetic mutations PKD1 PKD2 leads disrupted Ca+2 cAMP signaling, aberrant fluid secretion, uncontrolled proliferation, resulting tubular cystogenesis. Understanding molecular mechanisms underlying dysfunctions has opened door innovative therapeutic strategies, TRPV4 activators, CFTR inhibitors, calcimimetics, mitigate cyst growth preserve function. This review summarizes current knowledge on roles PKD pathophysiology, highlights interventions targeting identifies future research directions improving patient outcomes.

Language: Английский

Ciliary Ion Channels in Polycystic Kidney Disease DOI Creative Commons
Lubna A. Alshriem,

Raghad Buqaileh,

Qasim Alorjani

et al.

Cells, Journal Year: 2025, Volume and Issue: 14(6), P. 459 - 459

Published: March 19, 2025

Polycystic kidney disease (PKD) is the most common hereditary disorder that disrupts renal function and frequently progresses to end-stage disease. Recent advances have elucidated critical role of primary cilia ciliary ion channels, including transient receptor potential (TRP) cystic fibrosis transmembrane conductance regulator (CFTR), polycystin in pathogenesis PKD. While some channels primarily as chloride (e.g., CFTR), others regulate calcium (Ca+2) homeostasis. These are essential for cellular signaling maintaining normal architecture. Dysregulation these pathways due genetic mutations PKD1 PKD2 leads disrupted Ca+2 cAMP signaling, aberrant fluid secretion, uncontrolled proliferation, resulting tubular cystogenesis. Understanding molecular mechanisms underlying dysfunctions has opened door innovative therapeutic strategies, TRPV4 activators, CFTR inhibitors, calcimimetics, mitigate cyst growth preserve function. This review summarizes current knowledge on roles PKD pathophysiology, highlights interventions targeting identifies future research directions improving patient outcomes.

Language: Английский

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