## Abstract Individuals with tuberous sclerosis complex (TSC) develop central nervous system abnormalities that may reflect astrocyte dysfunction. In an effort to model astrocyte dysfunction in TSC, we generated mice lacking __Tsc1__ (hamartin) expression in astrocytes and demonstrated that __Tsc1_
Non-canonical functions of the tuberous sclerosis complex-Rheb signalling axis
β Scribed by Nicole A. Neuman; Elizabeth Petri Henske
- Book ID
- 102187158
- Publisher
- John Wiley and Sons
- Year
- 2011
- Tongue
- English
- Weight
- 401 KB
- Volume
- 3
- Category
- Article
- ISSN
- 1757-4676
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β¦ Synopsis
Abstract
The protein products of the tuberous sclerosis complex (TSC) genes, TSC1 and TSC2, form a complex, which inhibits the small Gβprotein, Ras homolog enriched in brain (Rheb). The vast majority of research regarding these proteins has focused on mammalian Target of Rapamycin (mTOR), a target of Rheb. Here, we propose that there are clinically relevant functions and targets of TSC1, TSC2 and Rheb, which are independent of mTOR. We present evidence that such nonβcanonical functions of the TSCβRheb signalling network exist, propose a standard of evidence for these nonβcanonical functions, and discuss their potential clinical and therapeutic implications for patients with TSC and lymphangioleiomyomatosis (LAM).
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