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Targeting chloride transport in autosomal dominant polycystic kidney disease

François Jouret1, Olivier Devuyst2

  • 1Division of Nephrology, Department of Internal Medicine, ULiège Academic Hospital, Liège, Belgium,; Groupe Interdisciplinaire de Géno-protéomique Appliquée, Cardiovascular Sciences, ULiège Medical School, Liège, Belgium.

Cellular Signalling
|July 4, 2020
PubMed

Insights

Autosomal dominant polycystic kidney disease (ADPKD) involves cyst growth driven by fluid secretion. Targeting chloride transport via the CFTR channel offers a promising therapeutic strategy to slow kidney disease progression.

Area of Science:

  • Nephrology
  • Genetics
  • Molecular Biology

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder.
  • Fluid secretion by cyst-lining cells is a primary driver of cystogenesis in ADPKD.
  • Chloride secretion, mediated by the CFTR channel and cyclic AMP, is implicated in ADPKD fluid secretion.

Purpose of the Study:

  • To review the pathophysiology of fluid secretion in ADPKD.
  • To highlight the contributions of Jared Grantham and colleagues to understanding ADPKD.
  • To explore molecular mechanisms and potential therapeutic applications.

Main Methods:

  • Review of existing literature on ADPKD pathophysiology.
  • Analysis of studies on fluid and chloride transport in ADPKD.
  • Examination of molecular pathways involved in cystogenesis.

Main Results:

  • Fluid secretion in ADPKD is primarily driven by chloride transport.
  • The cystic fibrosis transmembrane conductance regulator (CFTR) channel plays a key role.
  • Elevated intracellular cyclic adenosine monophosphate stimulates fluid secretion.

Conclusions:

  • Understanding fluid and chloride transport is crucial for ADPKD research.
  • Targeting these mechanisms offers potential therapeutic strategies.
  • Preventing cyst volume expansion can slow ADPKD progression.

Keywords:
CFTREpithelial cellIon transportKidney cystVasopressin

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