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TRP channels and kidney disease: lessons from polycystic kidney disease

S Qamar1, M Vadivelu, R Sandford

  • 1Department of Medical Genetics, Cambridge Institute of Medical Research, Hills Road, Cambridge CB2 2XY, UK.

Biochemical Society Transactions
|January 20, 2007
PubMed

Insights

Mutations in the PKD2 gene, encoding transient receptor potential polycystin 2 (TRPP2), link TRP channel function to kidney cyst formation. TRPP2 regulates cellular processes through calcium signaling in the primary cilium.

Area of Science:

  • Molecular Biology
  • Cell Biology
  • Nephrology

Background:

  • Transient receptor potential (TRP) channel superfamily plays critical roles in cellular functions.
  • Mutations in disease-related genes provide key insights into channel function.
  • Autosomal dominant polycystic kidney disease (ADPKD) is linked to mutations in the PKD2 gene.

Purpose of the Study:

  • To investigate the role of the PKD2 gene and its encoded protein, TRPP2, in autosomal dominant polycystic kidney disease.
  • To understand the function of TRPP2 in mechanosensation and primary cilia in renal cystogenesis.

Main Methods:

  • Genetic analysis of disease-related mutations.
  • Functional characterization of the polycystin complex.
  • Analysis of TRPP2 expression and localization.

Main Results:

  • PKD2 mutations are implicated in ADPKD, highlighting TRP channel involvement in renal cyst formation.
  • TRPP2 forms a complex with polycystin-1 in the renal primary cilium, responding to mechanical stimuli like flow.
  • TRPP2 regulates both extracellular calcium influx and intracellular calcium release, and also modulates intracellular calcium signaling from the endoplasmic reticulum/sarcoplasmic reticulum.

Conclusions:

  • TRPP2 is a crucial component of the polycystin complex involved in renal mechanosensation.
  • TRPP2's regulation of intracellular calcium signaling pathways is vital for normal kidney function and implicated in cyst formation.
  • TRPP2's multifaceted role in calcium homeostasis underscores its importance in cellular processes and disease pathogenesis.

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