Gigas, a Drosophila homolog of tuberous sclerosis gene product-2, regulates the cell cycle
Gigas, a Drosophila homolog of tuberous sclerosis gene product-2, regulates the cell cycle
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DOI：
10.1016/s0092-8674(00)80657-1
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发表时间：
1999-02-19
期刊：
影响因子：
64.5
通讯作者：
Rubin, GM
中科院分区：
文献类型：
--
作者：
Ito, N;Rubin, GM
结节性硬化症(TSC)是一种常染色体显性遗传性疾病，导致良性肿瘤的广泛发展，通常含有巨细胞。我们证明，果蝇基因Gigas编码TSC2的同源基因，TSC2基因在一半的TSC患者中发生了突变。在想象盘中诱导的巨细胞突变细胞克隆正常分化，产生成体结构。然而，这些克隆中的细胞被放大并重复S期，而不进入M期。我们的结果表明，TSC紊乱可能是细胞周期控制的潜在缺陷所致。我们还鉴定了TSC1的一个果蝇同源物。
Tuberous sclerosis complex (TSC) is an autosomal dominant disorder leading to the widespread development of benign tumors that often contain giant cells. We show that the Drosophila gene gigas encodes a homolog of TSC2, a gene mutated in half of TSC patients. Clones of gigas mutant cells induced in imaginal discs differentiate normally to produce adult structures. However, the cells in these clones are enlarged and repeat S phase without entering M phase. Our results suggest that the TSC disorder may result from an underlying defect in cell cycle control. We have also identified a Drosophila homolog of TSC1.