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The mTOR pathway is activated in human autosomal-recessive polycystic kidney disease

Jan Ulrich Becker1, Anabelle Opazo Saez, Klaus Zerres

  • 1Institute of Pathology and Neuropathology, University Hospital of Essen, University Duisburg-Essen, Essen, Germany.

Kidney & Blood Pressure Research
|May 13, 2010
PubMed

Insights

The mechanistic target of rapamycin (mTOR) pathway may be activated in autosomal-recessive polycystic kidney disease (AR PKD). This suggests mTOR molecules could be a target for slowing cyst development in AR PKD.

Area of Science:

  • Nephrology
  • Molecular Biology
  • Cell Signaling

Background:

  • Autosomal dominant (AD) polycystic kidney disease (PKD) shows inappropriate mTOR pathway activation.
  • The role of the mTOR pathway in autosomal recessive (AR) PKD, a pediatric cystic disease, remains unclear.

Purpose of the Study:

  • To investigate the activation status of the mTOR pathway in autosomal recessive (AR) PKD.

Main Methods:

  • Evaluation of mTOR pathway molecule expression in kidney and liver samples from AR PKD patients and controls.
  • Utilized monoclonal antibodies for phosphorylated proteins: pmTOR, pS6K, p4E-BP1, peIF4G, and phospho-tuberin/TSC2.

Main Results:

  • Strong mTOR expression observed in renal cyst-lining cells and bile ducts in AR PKD.
  • Differential S6K and 4E-BP1 expression patterns noted in AR PKD tissues compared to controls.
  • eIF4G immunostaining was present in AR PKD bile ducts but absent in controls; Tuberin/TSC2 was negative in all samples.

Conclusions:

  • Findings suggest potential mTOR pathway activation in AR PKD.
  • mTOR pathway molecules may serve as therapeutic targets to mitigate cystogenesis in AR PKD.
Abstract

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