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Citation
Pan, D., Dong, J., Zhang, Y., Gao, X. (2004). Tuberous sclerosis complex: from Drosophila to human disease.  Trends Cell Biol. 14(2): 78--85.
FlyBase ID
FBrf0175159
Publication Type
Review
Abstract

Tuberous sclerosis complex (TSC) is a human syndrome characterized by a widespread development of benign tumors. This disease is caused by mutations in the TSC1 or TSC2 tumor suppressor genes; the molecular mechanisms underlying the activity of these have long been elusive. Recent studies of Drosophila and mammalian cells demonstrate that the TSC1-TSC2 complex functions as GTPase activating protein against Rheb - a Ras-like small GTPase, which in turn regulates TOR signaling in nutrient-stimulated cell growth. These findings provide a new paradigm for how proteins involved in nutrient sensing could function as tumor suppressors and suggest novel therapeutic targets against TSC. Here, we review these exciting developments with an emphasis on Drosophila studies and discuss how Drosophila can be a powerful model system for an understanding of the molecular mechanisms of the activity of human disease genes.

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    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Trends Cell Biol.
    Title
    Trends in Cell Biology
    Publication Year
    1991-
    ISBN/ISSN
    0962-8924
    Data From Reference
    Genes (13)
    Human Disease Models (3)