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Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
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Recent advances in understanding ion transport mechanisms in polycystic kidney disease

Anastasia V Sudarikova1, Valeriia Y Vasileva1, Regina F Sultanova2

  • 1Institute of Cytology, Russian Academy of Sciences, St. Petersburg, Russia.

Clinical Science (London, England : 1979)
|November 9, 2021
PubMed

Insights

This review explores how electrolyte transport mechanisms contribute to inherited kidney diseases like autosomal dominant (ADPKD) and autosomal recessive (ARPKD) polycystic kidney disease, focusing on cyst development.

Area of Science:

  • Nephrology
  • Molecular Biology
  • Genetics

Background:

  • Polycystic kidney diseases (PKD), including autosomal dominant (ADPKD) and autosomal recessive (ARPKD) forms, are severe inherited renal disorders.
  • Understanding the underlying molecular mechanisms of cystogenesis is crucial for developing effective treatments.

Purpose of the Study:

  • To review recent advances in electrolyte transport mechanisms relevant to ADPKD and ARPKD pathogenesis.
  • To discuss the role of intracellular calcium handling and sodium transport in cyst formation.

Main Methods:

  • Literature review of recent research on electrolyte transport in polycystic kidney disease.
  • Focus on key proteins and cellular machinery involved in ion transport and fluid secretion.

Main Results:

  • Electrolyte transport, particularly calcium and sodium handling, plays a significant role in cystogenesis in PKD.
  • Proteins like polycystins, fibrocystin, TRPV4 channels, and ENaC are implicated in disease development.
  • Chloride-dependent fluid secretion contributes to cystic fluid accumulation.

Conclusions:

  • Advances in understanding electrolyte transport offer new insights into PKD.
  • Targeting ion transport mechanisms presents promising therapeutic avenues for polycystic kidney disease.
  • Further research into potassium transport and electrolyte handling is warranted.

Keywords:
Electrolyte transportIon channelsKidneyPolycystic Kidney DiseaseRenal Physiologypurinergic signaling

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