FAD-linked sulfhydryl oxidase ALR (GFER)

Isoform 1: FAD-dependent sulfhydryl oxidase that regenerates the redox-active disulfide bonds in CHCHD4/MIA40, a chaperone essential for disulfide bond formation and protein folding in the mitochondrial intermembrane space. The reduced form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with GFER/ERV1, leading to regeneration of the essential disulfide bonds in CHCHD4/MIA40, while GFER/ERV1 becomes oxidized by donating electrons to cytochrome c or molecular oxygen.

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