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Table 1 a GA1 glutaric acidemia type 1, MMA methylmalonic acidemia, PA propionic acidemia b IV indicates a patient whom was taken off oral carnitine (prior chronic PO dose is listed) and instead was on intravenous carnitine replacement for 10 days prior to sampling as a result of illness and inpatient hospitalization c Antibiotic treatments within 30 days of sampling are listed

Further in vivo studies showed that in the hearts of MI/R rats, the high-dose PV group (400 mg/kg/d) significantly upregulated the protein level of GPX4, and this upregulatory effect disappeared in Nrf2 knockout mice, indicating that the effect of PV in upregulating the GPX4 protein level is dependent on Nrf2 [182]