Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
EMBO Mol Med. 2011 Apr;3(4):189-200. doi: 10.1002/emmm.201100131. Epub 2011 Mar 16.
The protein products of the tuberous sclerosis complex (TSC) genes, TSC1 and TSC2, form a complex, which inhibits the small G-protein, 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 non-canonical functions of the TSC-Rheb signalling network exist, propose a standard of evidence for these non-canonical functions, and discuss their potential clinical and therapeutic implications for patients with TSC and lymphangioleiomyomatosis (LAM).
结节性硬化症复合物（TSC）基因的蛋白产物 TSC1 和 TSC2 形成复合物，抑制富含脑的 Ras 同源物（Rheb）的小 G 蛋白。这些蛋白的绝大多数研究都集中在雷帕霉素靶蛋白（mTOR）上，这是 Rheb 的靶点。在这里，我们提出 TSC1、TSC2 和 Rheb 存在与 mTOR 无关的具有临床相关性的功能和靶点。我们提出了存在 TSC-Rheb 信号网络的这种非典型功能的证据，提出了这些非典型功能的证据标准，并讨论了它们对 TSC 和淋巴管平滑肌瘤病（LAM）患者的潜在临床和治疗意义。
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