Recent Advances in Understanding Ion Transport Mechanisms in Polycystic Kidney Disease

Overview

Journal Clin Sci (Lond)

Specialties Biochemistry
General Medicine
Science

Date 2021 Nov 9

PMID 34751394

Citations 5

Authors

Anastasia V Sudarikova

Valeriia Y Vasileva

Regina F Sultanova

Daria V Ilatovskaya

Affiliations

Soon will be listed here.

Abstract

This review focuses on the most recent advances in the understanding of the electrolyte transport-related mechanisms important for the development of severe inherited renal disorders, autosomal dominant (AD) and recessive (AR) forms of polycystic kidney disease (PKD). We provide here a basic overview of the origins and clinical aspects of ARPKD and ADPKD and discuss the implications of electrolyte transport in cystogenesis. Special attention is devoted to intracellular calcium handling by the cystic cells, with a focus on polycystins and fibrocystin, as well as other calcium level regulators, such as transient receptor potential vanilloid type 4 (TRPV4) channels, ciliary machinery, and purinergic receptor remodeling. Sodium transport is reviewed with a focus on the epithelial sodium channel (ENaC), and the role of chloride-dependent fluid secretion in cystic fluid accumulation is discussed. In addition, we highlight the emerging promising concepts in the field, such as potassium transport, and suggest some new avenues for research related to electrolyte handling.

Citing Articles

Electrolyte and metabolite composition of cystic fluid from a rat model of ARPKD.

Klemens C, Fedoriuk M, Semenikhina M, Stefanenko M, Zietara A, Levchenko V Commun Biol. 2025; 8(1):230.

PMID: 39948436 PMC: 11825955. DOI: 10.1038/s42003-025-07631-w.

Novel Potential Therapeutic Targets in Autosomal Dominant Polycystic Kidney Disease from the Perspective of Cell Polarity and Fibrosis.

Ahn Y, Park J Biomol Ther (Seoul). 2024; 32(3):291-300.

PMID: 38589290 PMC: 11063481. DOI: 10.4062/biomolther.2023.207.

Kinesin family member 3A induces related diseases via wingless-related integration site/β-catenin signaling pathway.

Liu S, He Y, Li S, Gao X, Yang F Sci Prog. 2023; 106(1):368504221148340.

PMID: 36594221 PMC: 10358705. DOI: 10.1177/00368504221148340.

Modulation of P2X receptor activity by ivermectin and 5-BDBD has no effect on the development of ARPKD in PCK rats.

Xu B, Nikolaienko O, Levchenko V, Choubey A, Isaeva E, Staruschenko A Physiol Rep. 2022; 10(21):e15510.

PMID: 36353932 PMC: 9647406. DOI: 10.14814/phy2.15510.

Ion channels as a therapeutic target for renal fibrosis.

Yan P, Ke B, Fang X Front Physiol. 2022; 13:1019028.

PMID: 36277193 PMC: 9581181. DOI: 10.3389/fphys.2022.1019028.

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