Tuberous sclerosis complex: From Drosophila to human disease

Duojia Pan, Jixin Dong, Yong Zhang, Xinsheng Gao

Research output: Contribution to journalReview articlepeer-review

130 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)78-85
Number of pages8
JournalTrends in Cell Biology
Volume14
Issue number2
DOIs
StatePublished - Feb 2004

ASJC Scopus subject areas

  • Cell Biology

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