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


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.

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Compartment endoplasmic reticulum lumen
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PhysicalEntity Activity Active Units
SUMF1 oxidoreductase activity (0016491)  
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Literature References
pubMedId Title Journal Year
15657036 Molecular characterization of the human Calpha-formylglycine-generating enzyme J Biol Chem 2005
12757706 The multiple sulfatase deficiency gene encodes an essential and limiting factor for the activity of sulfatases Cell 2003
14563551 The human SUMF1 gene, required for posttranslational sulfatase modification, defines a new gene family which is conserved from pro- to eukaryotes Gene 2003
12757705 Multiple sulfatase deficiency is caused by mutations in the gene encoding the human C(alpha)-formylglycine generating enzyme Cell 2003
8681943 The evolutionary conservation of a novel protein modification, the conversion of cysteine to serinesemialdehyde in arylsulfatase from Volvox carteri Eur J Biochem 1996
9699462 A novel protein modification generating an aldehyde group in sulfatases: its role in catalysis and disease Bioessays 1998
16368756 A general binding mechanism for all human sulfatases by the formylglycine-generating enzyme Proc Natl Acad Sci U S A 2006
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Orthologous events