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BRUP-1 activates the Nrf2-HO-1 axis by directly inhibiting the interaction between Nrf2 and Kelch-like ECH-associated protein 1 (Keap1), and increases the production of BR, thereby providing an approach more in line with physiological mechanisms ( In summary, clinical trial design must take into account the dual role of bilirubin, including formulating a rigorous dose escalation scheme, stratifying patients based on UGT1A1 genotype, and simultaneously monitoring signs of both efficacy and bilirubin neurotoxicity

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In FRDA, however, this defense system breaks down at the genetic level

And now I feel normal again