1.8.3.7: formylglycine-generating enzyme
This is an abbreviated version!
For detailed information about formylglycine-generating enzyme, go to the full flat file.
Word Map on EC 1.8.3.7
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1.8.3.7
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sulfatases
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metachromatic
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mucopolysaccharidosis
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leukodystrophy
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ichthyosis
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antibody-drug
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ultra-rare
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morquio
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medicine
- 1.8.3.7
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sulfatases
-
metachromatic
- mucopolysaccharidosis
- leukodystrophy
-
ichthyosis
-
antibody-drug
-
ultra-rare
- morquio
- medicine
Reaction
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
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General Information
General Information on EC 1.8.3.7 - formylglycine-generating enzyme
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malfunction
metabolism
physiological function
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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
malfunction
enzyme inactivity results in multiple sulfatase deficiency, a fatal autosomal recessive syndrome
malfunction
multiple sulfatase deficiency is caused by mutations in the SUMF1 gene encoding the formylglycine-generating enzyme
malfunction
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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
metabolism
the enzyme posttranslationally activates sulfatases by generating formylglycine in their catalytic sites
the enzyme is required for the posttranslational activation of type I sulfatases by oxidation of an active-site cysteine to Ca-formylglycine
physiological function
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the enzyme is required for the posttranslational activation of type I sulfatases by oxidation of an active-site cysteine to Ca-formylglycine
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