产品说明
Tuberoussclerosiscomplex(TSC)isanautosomaldominanttumorsyndromecausedbymutationsineitheroftheTSC1orTSC2tumorsuppressorgenes.TheproductsofthesegenesformaproteincomplexthatindirectlydecreasesthesignalingofthemammalianTargetofRapamycin(TOR),anevolutionarilyconservedserine/threoninekinasethatregulatescellgrowthandcellcyclethroughitsABIlitytointegratesignalsfromnutrientlevelsandgrowthfactors.TORactivityisstimulatedbyRheb,amemberoftheRassuperfamilyofG-proteins,whentheGTP/GDPratioboundtoRhebishigh.ImmunoprecipitatedTSC1/TSC2hasbeenshowntostimulateRhebGTPaseactivityinvitro,suggestingthattheTSC1/TSC2complexdecreasestheabilityofRhebtostimulateTORactivity.ThisissupportedbyexperimentsshowingthatoverexpressionofTSC1andTSC2resultsinasignificantdecreaseintheGTP/GDPratioboundtoRhebandtheinhibitionofcellgrowth.AtleastthreeisoformsofTSC2exist.
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