Reactome: A Curated Pathway Database

SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases (R-HSA-1614362)

Species Homo sapiens

Summation

The sulfatase-modifying factor 1 (SUMF1, also called C-alpha-formylglycine-generating enzyme, FGE) (Preusser-Kunze et al. 2005, Cosma et al. 2003, Landgrebe et al. 2003) oxidises the critical cysteine residue in arylsulfatases to an active site 3-oxoalanine residue thus confering sulfatase activity (Roeser et al. 2006). Defects in SUMF1 cause multiple sulfatase deficiency (MSD) (MIM:272200), an impairment of arylsulfatase activity due to defective post-translational modification of the cysteine residue (Cosma et al. 2003, Dierks et al, 2003). This post-translational modification is thought to be highly conserved in eukaryotes (Selmer et al. 1996, von Figura et al. 1998). SUMF1 is active as either a monomer or a homodimer. A monomer is described in this reaction.

Locations in the PathwayBrowser
Additional Information
Compartment endoplasmic reticulum lumen
Components of this entry
Input entries
Output entries
Catalyst Activity
PhysicalEntity Activity Active Units
SUMF1 oxidoreductase activity (0016491)
This entry is regulated by
Regulation type Name
NegativeRegulation