The activation of arylsulfatases

Stable Identifier
R-HSA-1663150
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Type
Pathway
Species
Homo sapiens
ReviewStatus
5/5
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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
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