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SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases |
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| Definitions |
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. Reviewed: D'Eustachio, P, 2012-05-14 Authored: Jassal, B, 2011-09-28 Edited: Jassal, B, 2011-09-28 |
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| ID |
http://purl.obolibrary.org/obo/HINO_0004193 |
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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. Reviewed: D'Eustachio, P, 2012-05-14 Authored: Jassal, B, 2011-09-28 Edited: Jassal, B, 2011-09-28 |
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| definition source |
Pubmed16368756 Pubmed12757705 Reactome, http://www.reactome.org Pubmed9699462 Pubmed12757706 Pubmed14563551 Pubmed8681943 Pubmed15657036 |
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http://purl.obolibrary.org/obo/CHEBI_16908 |
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SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases |
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| prefixIRI |
HINO:0004193 |
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| prefLabel |
SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases |
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| seeAlso |
Reactome Database ID Release 431614362 ReactomeREACT_121125 EC Number: 1 |
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