InterPro domain: IPR039799

This entry contains FAD-dependent sulfhydryl oxidases, including ALR from animals and Erv from plants and fungi. ALR generates the disulfide bonds in CHCHD4/MIA40, which in turn is a chaperone required for disulfide bond formation and protein folding in the mitochondrial intermembrane space [ 1 ]. ERV1 from Arabidopsis is required for the import and folding of small cysteine-containing proteins in the mitochondrial intermembrane space [ 2 ]. Erv2 from Saccharomyces cerevisiae catalyzes disulfide bond formation in the endoplasmic reticulum lumen [ 3 ].

1. Augmenter of liver regeneration: substrate specificity of a flavin-dependent oxidoreductase from the mitochondrial intermembrane space. Biochemistry 48, 4828-37
2. Unique features of plant mitochondrial sulfhydryl oxidase. J. Biol. Chem. 279, 20002-8
3. A flavoprotein oxidase defines a new endoplasmic reticulum pathway for biosynthetic disulphide bond formation. Nat. Cell Biol. 3, 874-82