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Hamartin and tuberin modulate gene transcription via β-catenin

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Summary

Tuberous sclerosis, neurological genetic disorder characterized by the formation of benign tumors or hamartomas in multiple organ systems, is recently getting much attention. Numerous papers describe still-not-fully-explained pathogenesis of the disease. Studies on tuberous sclerosis allowed identification of two tumor suppressor genes, TSC1 and TSC2, encoding proteins implicated in the disease: hamartin and tuberin, respectively. The importance of these proteins is confirmed by their ubiquitous character and by the fact that TSC1/TSC2 complex is involved in the regulation of the activity of mTOR, a master controller of protein translation. Thus, the meaning of hamartin and tuberin goes far beyond tuberous sclerosis. As far as the influence of the TSC1/TSC2 complex on protein translation is well described in numerous reviews, little attention is drawn to the recently discovered role of the TSC1/TSC2 complex in gene transcription via the WNT signaling pathway. The present paper focuses on recent developments documenting the role of hamartin and tuberin in the WNT pathway.

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Authors and Affiliations

  1. Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, ul. Chalubinkiego 5, 02-004, Warsaw, Poland

    Jaroslaw Jozwiak & Pawel Wlodarski

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  1. Jaroslaw Jozwiak
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  2. Pawel Wlodarski
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Correspondence to Jaroslaw Jozwiak.

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Jozwiak, J., Wlodarski, P. Hamartin and tuberin modulate gene transcription via β-catenin. J Neurooncol 79, 229–234 (2006). https://doi.org/10.1007/s11060-006-9134-0

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  • DOI: https://doi.org/10.1007/s11060-006-9134-0

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