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Glucosamine (N-acetyl)-6-sulfatase
Symbols GNS ; G6S
External IDs OMIM607664 HomoloGene1568 GeneCards: GNS Gene
EC number
Species Human Mouse
Entrez 2799 75612
Ensembl ENSG00000135677 ENSMUSG00000034707
UniProt P15586 Q8BFR4
RefSeq (mRNA) NM_002076 NM_029364
RefSeq (protein) NP_002067 NP_083640
Location (UCSC) Chr 12:
65.11 – 65.15 Mb
Chr 10:
121.37 – 121.4 Mb
PubMed search [1] [2]
EC number
CAS number 60320-99-2
IntEnz IntEnz view
ExPASy NiceZyme view
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / EGO

N-acetylglucosamine-6-sulfatase also known as glucosamine (N-acetyl)-6-sulfatase is an enzyme that in humans is encoded by the GNS gene.[1] This enzyme is deficient in Sanfilippo Syndrome type IIId.[2][3][4] This enzyme catalyses the following chemical reaction:

Hydrolysis of the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of heparan sulfate and keratan sulfate


N-acetylglucosamine-6-sulfatase is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate.[1]

Clinical significance[edit]

Deficiency of this enzyme results in the accumulation of undergraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.[1]


The systematic name of this enzyme is "N-acetyl-D-glucosamine-6-sulfate 6-sulfohydrolase". Other accepted names include:

  • N-acetylglucosamine-6-sulfatase,
  • glucosamine (N-acetyl)-6-sulfatase,
  • 2-acetamido-2-deoxy-D-glucose 6-sulfate sulfatase,
  • N-acetylglucosamine 6-sulfate sulfatase,
  • O,N-disulfate O-sulfohydrolase,
  • acetylglucosamine 6-sulfatase,
  • chondroitinsulfatase, and
  • glucosamine-6-sulfatase.


  1. ^ a b c "Entrez Gene: Glucosamine (N-acetyl)-6-sulfatase". 
  2. ^ Basner R, Kresse H, von Figura K (February 1979). "N-Acetylglucosamine-6-sulfate sulfatase from human urine". J. Biol. Chem. 254 (4): 1151–8. PMID 762121. 
  3. ^ Kresse H, Fuchs W, Glössl J, Holtfrerich D, Gilberg W (December 1981). "N-acetylglucosamine-6-sulfate by human β-N-acetylhexosaminidase A". J. Biol. Chem. 256 (24): 12926–32. PMID 6458607. 
  4. ^ Weissmann B, Chao H, Chow P (November 1980). "A glucosamine O,N-disulfate O-sulfohydrolase with a probable role in mammalian catabolism of heparan sulfate". Biochem. Biophys. Res. Commun. 97 (2): 827–33. doi:10.1016/0006-291x(80)90338-1. PMID 6451222. 

External links[edit]

This article incorporates text from the United States National Library of Medicine, which is in the public domain.