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N-acetylgalactosamine-6-sulfatase; galactose-6-sulfatase; chondroitin sulfatase; chondroitinase (GALNS)
Function:
- hydrolysis of the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate & of the D-galactose 6-sulfate units of keratan sulfate
Cofactor: binds 1 Ca+2 per subunit (putative)
Structure: belongs to the sulfatase family
Compartment: lysosome
Pathology:
- deficient in Morquio's disease type A (mucopolysaccharidosis-IVa)
General
Ca+2 binding protein
glycoprotein
sulfatase
Properties
SIZE: entity length = 522 aa
MW = 58 kD
COMPARTMENT: lysosome
MOTIF: signal sequence {1-26}
Ca+2-binding site
SITE: 39-39
Ca+2-binding site
SITE: 40-40
Ca+2-binding site
SITE: 79-79
N-glycosylation site {N204}
Ca+2-binding site
SITE: 288-288
Ca+2-binding site
SITE: 289-289
N-glycosylation site {N423}
Database Correlations
OMIM 253000
UniProt P34059
Pfam PF00884
ENZYME 3.1.6.4
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=GALNS
- UniProt :accession P34059