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Literature summary for 1.8.3.7 extracted from

  • Schlotawa, L.; Steinfeld, R.; von Figura, K.; Dierks, T.; Gaertner, J.
    Molecular analysis of SUMF1 mutations stability and residual activity of mutant formylglycine-generating enzyme determine disease severity in multiple sulfatase deficiency (2008), Hum. Mutat., 29, 205 .
    View publication on PubMed
Search for more literature for 1.8.3.7

Protein Variants

Protein Variants Comment Organism
A177P the mutant shows 0.6% of wild type activity Homo sapiens
A279V the mutant shows 22.9% of wild type activity Homo sapiens
R349W the mutant shows 0.5% of wild type activity Homo sapiens
W179S the mutant shows 2.7% of wild type activity Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
endoplasmic reticulum
-
 Homo sapiens 5783
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
a [sulfatase]-L-cysteine + O2 + a thiol Homo sapiens
-
 a [sulfatase]-3-oxo-L-alanine + hydrogen sulfide + a disulfide + H2O
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens Q8NBK3
-
-

Purification (Commentary)

Purification (Comment) Organism
MonoQ column chromatography Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
HT-1080 cell
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
a [sulfatase]-L-cysteine + O2 + a thiol
-
 Homo sapiens a [sulfatase]-3-oxo-L-alanine + hydrogen sulfide + a disulfide + H2O
-
 ?

Subunits

Subunits Comment Organism
? x * 38000, secreted enzyme, SDS-PAGE Homo sapiens
? x * 41000, full-length enzyme, SDS-PAGE Homo sapiens

Synonyms

Synonyms Comment Organism
FGE
-
 Homo sapiens
SUMF1
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction multiple sulfatase deficiency is caused by mutations in the SUMF1 gene encoding the formylglycine-generating enzyme Homo sapiens
metabolism the enzyme posttranslationally activates sulfatases by generating formylglycine in their catalytic sites Homo sapiens