| Autor: |
Fidalgo da Silva, Elizabeth, Ansari, Shora B., Maimaiti, Jiamila, Barnes, Elizabeth A., Kong-Beltran, Monica, Donoghue, Daniel J., Porter, Lisa A. |
| Zdroj: |
Cell Cycle; September 2011, Vol. 10 Issue: 18 p3129-3139, 11p |
| Abstrakt: |
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 G2/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. |
| Databáze: |
Supplemental Index |
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