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Description
Free intracellular cysteine normally prevents auto-oxidation and inhibition of hypoxia-inducible factor prolyl hydroxylase 2 (EgIN1), which targets hypoxia inducible factor 1 (HIF1) for degradation

Functional Improvement: Improvement in function has been observed, especially in younger patients with isolated defects

Antonoff MB, Kui N, Sun R, Deboever N, Hofstetter W, Mehran RJ, Morris VK, Rice DC , Swisher SG, Vaporciyan AA, Walsh GL, Rajaram R

These mechanisms are described as research areas, not clinical outcomes
