View source for N-acetylglucosamine-6-sulfatase

{{Infobox_gene}}
{{Infobox enzyme
| Name = N-acetylglucosamine-6-sulfatase
| EC_number = 3.1.6.14
| CAS_number = 60320-99-2
| GO_code = GO:0008449
| image = 
| width = 
| caption = 
}}
'''N-acetylglucosamine-6-sulfatase''' also known as '''glucosamine (N-acetyl)-6-sulfatase''' is an [[enzyme]] that in humans is encoded by the GNS [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: Glucosamine (N-acetyl)-6-sulfatase | url = https://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=retrieve&list_uids=2799 }}</ref> This enzyme is deficient in [[Sanfilippo Syndrome]] type IIId.<ref name="pmid762121">{{cite journal |vauthors=Basner R, Kresse H, von Figura K | title = ''N''-Acetylglucosamine-6-sulfate sulfatase from human urine | journal = J. Biol. Chem. | volume = 254 | issue = 4 | pages = 1151–8 |date=February 1979 | pmid = 762121 | doi = | url = }}</ref><ref name="pmid6458607">{{cite journal |vauthors=Kresse H, Fuchs W, Glössl J, Holtfrerich D, Gilberg W | title = ''N''-acetylglucosamine-6-sulfate by human &beta;-''N''-acetylhexosaminidase A | journal = J. Biol. Chem. | volume = 256 | issue = 24 | pages = 12926–32 |date=December 1981 | pmid = 6458607 | doi = | url = }}</ref><ref name="pmid6451222">{{cite journal |vauthors=Weissmann B, Chao H, Chow P | title = A glucosamine O,''N''-disulfate ''O''-sulfohydrolase with a probable role in mammalian catabolism of heparan sulfate | journal = Biochem. Biophys. Res. Commun. | volume = 97 | issue = 2 | pages = 827–33 |date=November 1980 | pmid = 6451222 | doi = 10.1016/0006-291x(80)90338-1| url = }}</ref> This enzyme [[catalysis|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]].<ref name="entrez"/>

== 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]].<ref name="entrez"/>

== Nomenclature ==

The [[List of enzymes|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 ==
{{reflist}}

==External links==
* {{MeshName|N-acetylglucosamine-6-sulfatase}}

{{NLM content}}

{{Glycosaminoglycan catabolism}}
{{Esterases}}
{{Enzymes}}
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[[Category:EC 3.1.6]]


{{3.1-enzyme-stub}}