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Cell Cycle September 15, 2011; 10 (18): 3129-39.
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The tumor suppressor tuberin regulates mitotic onset through the cellular localization of cyclin B1.

Fidalgo da Silva E , Ansari SB , Maimaiti J , Barnes EA , Kong-Beltran M , Donoghue DJ , Porter LA .

Tuberous sclerosis is a multi-organ disorder characterized by the formation of benign tumors, called hamartomas, which affects more than 1 million people worldwide. The syndrome is initiated by a mutation in one of two tumor suppressor genes, TSC1 or TSC2, that encode for the proteins hamartin and tuberin, respectively. Herein, we demonstrate that tuberin binds and regulates the G 2/M cyclin, cyclin B1. We have determined that this binding region encompasses a mutational hotspot within tuberin that is implicated in some of the most severe cases of TS. Mimicking a mutation found in a subset of patients with tuberous sclerosis, we found a significant reduction in the binding between tuberin and cyclin B1. Functionally, our data supports that tuberin plays a role in regulating the cellular localization of cyclin B1. These results demonstrate a novel and clinically relevant mechanism, where tuberin functions in mitotic onset.

PubMed ID: 21900748
Article link: Cell Cycle

Species referenced: Xenopus
Genes referenced: ccnb1 tsc1 tsc2