Reactome | SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases

SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases

Stable Identifier

R-HSA-1614362

Type

Reaction [transition]

Species

Homo sapiens

Compartment

endoplasmic reticulum lumen

ReviewStatus

5/5

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SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases

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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 formylglycine (3-oxoalanine) residue thus conferring 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.

Literature References

PubMed ID	Title	Journal	Year
14563551	The human SUMF1 gene, required for posttranslational sulfatase modification, defines a new gene family which is conserved from pro- to eukaryotes Landgrebe, J, Dierks, T, Schmidt, B, von Figura, K	Gene	2003
12757706	The multiple sulfatase deficiency gene encodes an essential and limiting factor for the activity of sulfatases Cosma, MP, Pepe, S, Annunziata, I, Newbold, RF, Grompe, M, Parenti, G, Ballabio, A	Cell	2003
9699462	A novel protein modification generating an aldehyde group in sulfatases: its role in catalysis and disease von Figura, K, Schmidt, B, Selmer, T, Dierks, T	Bioessays	1998
12757705	Multiple sulfatase deficiency is caused by mutations in the gene encoding the human C(alpha)-formylglycine generating enzyme Dierks, T, Schmidt, B, Borissenko, LV, Peng, J, Preusser, A, Mariappan, M, von Figura, K	Cell	2003
16368756	A general binding mechanism for all human sulfatases by the formylglycine-generating enzyme Roeser, D, Preusser-Kunze, A, Schmidt, B, Gasow, K, Wittmann, JG, Dierks, T, von Figura, K, Rudolph, MG	Proc Natl Acad Sci U S A	2006
15657036	Molecular characterization of the human Calpha-formylglycine-generating enzyme Preusser-Kunze, A, Mariappan, M, Schmidt, B, Gande, SL, Mutenda, K, Wenzel, D, von Figura, K, Dierks, T	J Biol Chem	2005
8681943	The evolutionary conservation of a novel protein modification, the conversion of cysteine to serinesemialdehyde in arylsulfatase from Volvox carteri Selmer, T, Hallmann, A, Schmidt, B, Sumper, M, von Figura, K	Eur J Biochem	1996