22.214.171.124: formylglycine-generating enzyme
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For detailed information about formylglycine-generating enzyme, go to the full flat file.
Word Map on EC 126.96.36.199 Reaction
a [sulfatase]-L-cysteine +
2 a thiol
a [sulfatase]-3-oxo-L-alanine +
hydrogen sulfide +
a disulfide H2O Synonyms
AtsB, C-alpha-formylglycine-generating enzyme 1, Calpha-formylglycine-generating enzyme, Calpha-formylglycine-generating enzyme 1, FGE, FGly-generating enzyme, formylglycine generating enzyme, sulfatase-modifying factor 1, SUMF1
General Information on EC 188.8.131.52 - formylglycine-generating enzyme
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enzyme dysfunction causes a lysosomal storage disorder known as multiple sulfatase deficiency, where all sulfatases either lack or suffer from a severely reduced, catalytic activity caused by incomplete generation of formylglycine
enzyme inactivity results in multiple sulfatase deficiency, a fatal autosomal recessive syndrome
multiple sulfatase deficiency is caused by mutations in the SUMF1 gene encoding the formylglycine-generating enzyme
structural distortions due to missense mutations in human formylglycine-generating enzyme lead to multiple sulfatase deficiency
enzyme overexpression stimulates the generation of catalytically active sulfatases
the enzyme posttranslationally activates sulfatases by generating formylglycine in their catalytic sites