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Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
07:35

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring

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Advances in the pathogenesis and treatment of polycystic kidney disease

Vishal Patel1, Renuka Chowdhury, Peter Igarashi

  • 1Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.

Current Opinion in Nephrology and Hypertension
|May 12, 2009
PubMed
Abstract

Insights

Polycystic kidney disease (PKD) stems from primary cilium defects, impacting kidney cell growth and leading to cyst formation. Promising therapies targeting these pathways are now in human clinical trials.

Area of Science:

  • Nephrology
  • Genetics
  • Cell Biology

Background:

  • Polycystic kidney disease (PKD) is the leading genetic cause of chronic kidney failure.
  • Mouse models are crucial for understanding PKD pathogenesis and testing therapeutics.

Purpose of the Study:

  • To review current understanding of PKD pathogenesis.
  • To highlight therapeutic targets and agents validated in preclinical models.

Main Methods:

  • Review of literature on PKD mouse models and ciliopathies.
  • Analysis of signaling pathways implicated in cystogenesis.
  • Summary of preclinical drug efficacy data.

Main Results:

  • PKD is a ciliopathy linked to primary cilium dysfunction and altered planar cell polarity.
  • Acute kidney injury exacerbates cyst formation in PKD models.
  • Several drugs, including tolvaptan and src inhibitors, show promise in animal models.

Conclusions:

  • Primary cilium abnormalities disrupt signaling, causing renal cyst formation.
  • Acute kidney injury may influence PKD progression variability.
  • Therapeutic agents validated in PKD models are advancing to human clinical trials.

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