Mutations in either gene impair this surveillance, allowing damaged mitochondria to persist and propagate oxidative stress (Franco et al., 2021
Stability is not judged by appearance alone
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NAC use is based on plotting APAP levels, on the Rumack-Matthew nomogram: Image from Wikipedia [Link is HERE] Intravenous NAC therapy includes: An initial dose of 150 mg/kg over 1 hour, A second dose of 50 mg/kg over 4 hours, And a final infusion of 100 mg/kg over 16 hours
Kinetic parameters were determined using LineweaverBurk plots using KaleidaGraph 4 (Synergy Software, least squares best fit of data and varying concentrations of one substrate while maintaining the concentration of other substrates