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The activation of arylsulfatases
Stable Identifier
R-HSA-1663150
DOI
10.3180/REACT_121036.1
Type
Pathway
Species
Homo sapiens
ReviewStatus
5/5
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Metabolism of proteins (Homo sapiens)
Post-translational protein modification (Homo sapiens)
Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activation (Homo sapiens)
The activation of arylsulfatases (Homo sapiens)
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Sulfatase activity requires a unique posttranslational modification (PTM) of a catalytic cysteine residue into a formylglycine. This modification is impaired in patients with multiple sulfatase deficiency (MSD) due to defects in the SUMF1 (sulfatase-modifying factor 1) gene responsible for this PTM. SUMF2 can inhibit the activity of SUMF1 thereby providing a mechanism for the regulation of sulfatase activation (Ghosh 2007, Diez-Roux & Ballabio 2005).
Literature References
PubMed ID
Title
Journal
Year
17558559
Human sulfatases: a structural perspective to catalysis
Ghosh, D
Cell Mol Life Sci
2007
16124866
Sulfatases and human disease
Diez-Roux, G
,
Ballabio, A
Annu Rev Genomics Hum Genet
2005
Participants
Events
SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
(Homo sapiens)
SUMF2 binds SUMF1, inhibiting SUMF1-mediated activation of arylsulfatases
(Homo sapiens)
Participates
as an event of
Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activation (Homo sapiens)
Orthologous Events
The activation of arylsulfatases (Bos taurus)
The activation of arylsulfatases (Canis familiaris)
The activation of arylsulfatases (Gallus gallus)
The activation of arylsulfatases (Mus musculus)
The activation of arylsulfatases (Rattus norvegicus)
The activation of arylsulfatases (Sus scrofa)
The activation of arylsulfatases (Xenopus tropicalis)
Authored
Jassal, B (2011-10-14)
Reviewed
D'Eustachio, P (2012-05-14)
Created
Jassal, B (2011-10-14)
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