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Fluid transport and cystogenesis in autosomal dominant polycystic kidney disease

Sara Terryn1, Anh Ho, Renaud Beauwens

  • 1Division of Nephrology, Université catholique de Louvain Medical School, B-1200, Brussels, Belgium.

Biochimica Et Biophysica Acta
|January 25, 2011
PubMed

Insights

Autosomal dominant polycystic kidney disease (ADPKD) involves cyst enlargement driven by chloride secretion. Understanding these mechanisms offers potential therapeutic targets for inherited nephropathy.

Area of Science:

  • Nephrology
  • Molecular Biology
  • Genetics

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder.
  • Cyst development in ADPKD involves cell proliferation, extracellular matrix changes, and fluid secretion.
  • Transepithelial chloride secretion, mediated by CFTR channels and basolateral transporters, is crucial for ADPKD cyst fluid accumulation.

Purpose of the Study:

  • To review the pathophysiology of fluid secretion in ADPKD cysts.
  • To explore the molecular mechanisms underlying cyst formation and fluid transport.
  • To discuss therapeutic strategies targeting these pathways.

Main Methods:

  • Review of morphological, physiological, and molecular studies.
  • Analysis of investigations in cellular and animal models.
  • Examination of therapeutic trials in ADPKD patients.

Main Results:

  • Fluid secretion in ADPKD cysts is primarily driven by chloride transport.
  • Increased intracellular cAMP levels, linked to calcium homeostasis and V2 receptor signaling, stimulate secretion.
  • Multiple studies highlight the role of CFTR and specific transporters in ADPKD pathogenesis.

Conclusions:

  • Understanding the molecular basis of fluid secretion is key to developing ADPKD treatments.
  • Targeting chloride secretion pathways presents a promising therapeutic avenue.
  • Further research in models and patients is crucial for effective interventions.

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