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Literature summary for 1.8.3.7 extracted from

  • Dierks, T.; Dickmanns, A.; Preusser-Kunze, A.; Schmidt, B.; Mariappan, M.; von Figura, K.; Ficner, R.; Rudolph, M.G.
    Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme (2005), Cell, 121, 541-552 .
    View publication on PubMed
Search for more literature for 1.8.3.7

Protein Variants

Protein Variants Comment Organism
A177P the mutant show reduced enzyme activity Homo sapiens
A279V the mutant show reduced enzyme activity Homo sapiens
A348P the mutant show reduced enzyme activity Homo sapiens
C218Y the mutant show reduced enzyme activity Homo sapiens
C336R the mutant show reduced enzyme activity Homo sapiens
C346W the mutant show reduced enzyme activity Homo sapiens
G247R the mutant show reduced enzyme activity Homo sapiens
G263V the mutant show reduced enzyme activity Homo sapiens
N259I the mutant show reduced enzyme activity Homo sapiens
P266L the mutant show reduced enzyme activity Homo sapiens
R224W the mutant show reduced enzyme activity Homo sapiens
R345C the mutant show reduced enzyme activity Homo sapiens
R349Q the mutant show reduced enzyme activity Homo sapiens
R349W the mutant show reduced enzyme activity Homo sapiens
R364C the mutant show reduced enzyme activity Homo sapiens
S155P the mutant show reduced enzyme activity Homo sapiens
S234R the mutant show reduced enzyme activity Homo sapiens
W179S the mutant show reduced enzyme activity Homo sapiens

Metals/Ions

Metals/Ions Comment Organism Structure
Ca2+ contains 2 Ca2+ ions Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
a [sulfatase]-L-cysteine + O(2) + a thiol Homo sapiens
-
 a [sulfatase]-3-oxo-L-alanine + hydrogen sulfide + a disulfide + H2O
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens Q8NBK3
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
glycoprotein N-glycosylation at Asn141 Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
a [sulfatase]-L-cysteine + O(2) + a thiol
-
 Homo sapiens a [sulfatase]-3-oxo-L-alanine + hydrogen sulfide + a disulfide + H2O
-
 ?

Synonyms

Synonyms Comment Organism
FGE
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction enzyme inactivity results in multiple sulfatase deficiency, a fatal autosomal recessive syndrome Homo sapiens