Tuberous sclerosis complex

Disease · 3 studies in the Atlas

Genetic disorder from TSC1/TSC2 loss causing constitutive mTOR activation and benign tumors in brain, kidney, and elsewhere. Everolimus shrinks these tumors (EXIST trials) - the clearest proof that blocking mTOR helps a human disease.

Evidence at a glance

TierWhat it meansStudies
BDirect human evidence2
DMechanistic / in vitro / review1

Studies

StudyYearTierFinding
BIS20132013BThe companion phase 3 RCT (n=118) to EXIST-1, targeting kidney tumors (angiomyolipomas) in tuberous sclerosis and LAM. Everolimus shrank them by >=50% in 42% of patients versus 0% on placebo. Together
FRA20132013BPhase 3 RCT (n=117) in tuberous sclerosis, the disease where mTOR is stuck ON by a genetic fault. Everolimus shrank brain tumors (SEGA) by >=50% in 35% of patients versus 0% on placebo. Because the ca
JOS20242024DmTORC1 activity oscillates across the cell cycle (lowest in mitosis/G1, highest in S/G2) via the TSC complex, independent of Akt/Mek-Erk; low mTORC1 in G1 sensitizes cells to autophagy induction from

Related entities

mTORC1 3Everolimus 2mTOR 1Autophagy 1Lymphangioleiomyomatosis 1

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