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SOD activity without adequate downstream peroxide removal is incomplete protection

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In the per-protocol population, the median [mean] annualized bleed rate (ABR) in the on-demand treatment arm was 41.5 [40.8] compared to 1.9 [3.7] while on a twice a week prophylaxis regimen

Gamma-glutamyl-cysteine is produced when the amino acids L-glutamate combine with L-cysteine in the presence of the enzyme glutamate-cysteine ligase, and this rate-limiting step is ATP-dependent