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Von hippel-lindau: a tumor suppressor links microtubules to ciliogenesis and cancer development

E Wolfgang Kuehn1, Gerd Walz, Thomas Benzing

  • 1Renal Division, University Hospital Freiburg, Freiburg, Germany.

Cancer Research
|May 19, 2007
PubMed

Insights

The von Hippel-Lindau (VHL) protein is crucial for cilia formation by orienting microtubules. Loss of VHL function in renal cell carcinoma disrupts this process, suggesting new tumor suppressor roles.

Area of Science:

  • Molecular Biology
  • Cell Biology
  • Oncology

Background:

  • Loss of the von Hippel-Lindau (VHL) tumor suppressor gene is implicated in familial and sporadic renal cell carcinoma.
  • The protein pVHL's known tumor suppressor function involves degrading hypoxia-inducible factors, but other roles are less understood.

Purpose of the Study:

  • To investigate the less-defined functions of pVHL beyond its role in hypoxia-inducible factor regulation.
  • To explore the connection between pVHL and cellular structures like cilia.

Main Methods:

  • Investigated the role of pVHL in cilia formation and microtubule orientation.
  • Examined the interaction of pVHL with PAR proteins in polarized epithelial cells.

Main Results:

  • p VHL is essential for the proper formation of cilia.
  • Loss of pVHL leads to incorrect orientation of newly formed microtubules and inhibits ciliogenesis.
  • pVHL interacts with PAR proteins to establish membrane domains and guide microtubule growth.

Conclusions:

  • The tumor suppressor protein pVHL plays a critical, previously unrecognized role in ciliogenesis.
  • Disruption of pVHL function in renal cell carcinoma may impact cellular pathways involving cilia and the cell cycle.

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