Source:http://www.reactome.org/biopax/48887BiochemicalReaction1946
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Authored: Jassal, B, 2011-09-28,
Edited: Jassal, B, 2011-09-28,
Reviewed: D'Eustachio, P, 2012-05-14,
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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biopax3:xref |
http://identifiers.org/pubmed/12757705,
http://identifiers.org/pubmed/12757706,
http://identifiers.org/pubmed/14563551,
http://identifiers.org/pubmed/15657036,
http://identifiers.org/pubmed/16368756,
http://identifiers.org/pubmed/8681943,
http://identifiers.org/pubmed/9699462,
urn:biopax:UnificationXref:REACTOME DATABASE ID_1614362,
urn:biopax:UnificationXref:REACTOME_REACT_121125_1
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SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
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1
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