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Mutation in TSC2 gene coding for Tuberin protein is known to lead to tuberous sclerosis in humans. Moreover, its gene product is believed to be a tumor suppressor and is able to stimulate specific GTPases playing important role in regulation of autophagy signaling pathway. Tuberin interacts with hamartin (TSC1) creating a heterodimeric complex that inhibit the mammalian target of rapamycin (mTOR) complex 1 (TORC1), which includes protein such as mTOR and Raptor. TORC1 further integrates mitogenic signals and nutrient availability with protein synthesis via substrates including p70 S6 kinase (S6K). Tuberin inhibits TORC1 via the Ras homologue Rheb, which is a key target of tuberin’s highly conserved guanosine triphosphatase activating protein (GAP) domain. Tuberin stimulates the conversion of Rheb–guanosine triphosphate (active) to Rheb–guanosine diphosphate (inactive), thereby inhibiting TORC1 and regulating autophagy process.

Fluorescent image of Hela cell stained with TSC2 Antibody(Cat#AM1919b). Hela cells were fixed with 4% PFA (20 min), permeabilized with Triton X-100 (0.1%, 10 min), then incubated with TSC2 primary antibody (1:25, 1 h at 37°C). For secondary antibody, Alexa Fluor® 488 conjugated donkey anti-rabbit antibody (green) was used (1:400, 50 min at 37°C.Cytoplasmic actin was counterstained with Alexa Fluor® 555 (red) conjugated Phalloidin (7units/ml, 1 h at 37°C). TSC2 is localized to microtubules in cytoplasm.

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Immunity Source (Host)
Available staus
TSC2 ()