N-acetylglucosamine-6-sulfatase (glucosamine-6-sulfatase, GNS)
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Function
- lysosomal enzyme that hydrolyzes the N-acetyl-D-glucosamine 6-sulfate units of heparan sulfate & keratan sulfate
- form A (78 kD) is processed by internal peptidase cleavage to a 32 kD N-terminal species (form B) & a 48 kD C-terminal species
Cofactor: binds 1 Ca+2 per subunit (putative)
Structure
belongs to the sulfatase family
Compartment
Pathology
- deficient in Sanfillipo D syndrome. [See sulfatase for PROSITE entry]
More general terms
References
- ↑ Textbook of Biochemistry with Clinical Correlations, 3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 383
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=GNS
- ↑ UniProt http://www.uniprot.org/uniprot/P15586.html