N-acetylglucosamine-6-sulfatase

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

Function

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

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]

Nomenclature

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.

References

↑ ^1.0 ^1.1 ^1.2 “Entrez Gene: Glucosamine (N-acetyl)-6-sulfatase”.
↑ 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.
↑ 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.
↑ 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

N-acetylglucosamine-6-sulfatase at the US National Library of Medicine Medical Subject Headings (MeSH)

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

[entrez-1] 1.0 ^1.1 ^1.2 “Entrez Gene: Glucosamine (N-acetyl)-6-sulfatase”.

[pmid762121-2] 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.

[pmid6458607-3] 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.

[pmid6451222-4] 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.

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