The Activation of the Transcription Factor NRF2 in Epithelial Cells Lining the Kidney Cysts in Tuberous Sclerosis Complex (TSC): The Interplay of Fumarate Hydratase 1, KEAP1, and NRF2 in Kidney Cystogenesis

Abstract Background. Tuberous Sclerosis Complex (TSC) is caused by inactivating mutations in either the TSC1 or TSC2 genes, leading to activation of the mammalian target of rapamycin complex 1 (mTORC1) and unhindered cell growth and proliferation. The epithelium of TSC renal cysts in both mice and humans is composed of proliferating A-intercalated (A-IC) cells. The exact molecular mechanism of…

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