Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme

Formylglycine (FGly)-generating enzyme (FGE) is a single-domain monomer with a surprising paucity of secondary structure and adopts a unique fold based on the crystal structure. FGE is localized in the endoplasmic reticulum (ER) and interacts with and modifies the unfolded form of newly synthesized sulfatases.