N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate

Stable Identifier
R-HSA-1638032
Type
Reaction [transition]
Species
Homo sapiens
Compartment
lysosomal lumen
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

N-acetylglucosamine 6-sulfatase (GNS) is a lysosomal enzyme which degrades glycosaminoglycans such as heparan sulfate and keratan sulfate. GNS shows strong sequence similarity to other sulphatases such as the family of arylsulfatases and the conversion to 3-oxo-alanine (formylglycine, FGly) of a cysteine residue is critical for catalytic activity, based on this similarity (Robertson et al. 1992, Robertson et al. 1988). Defects in GNS are the cause of mucopolysaccharidosis type IIID (MPSIIID, MIM:252940), also called Sanfilippo D syndrome (Valstar et al. 2010).

Literature References
PubMed ID Title Journal Year
1463457 A cDNA clone for human glucosamine-6-sulphatase reveals differences between arylsulphatases and non-arylsulphatases

Robertson, DA, Freeman, C, Morris, CP, Hopwood, JJ

Biochem J 1992
20232353 Mucopolysaccharidosis type IIID: 12 new patients and 15 novel mutations

Valstar, MJ, Bertoli-Avella, AM, Wessels, MW, Ruijter, GJ, de Graaf, B, Olmer, R, Elfferich, P, Neijs, S, Kariminejad, R, Suheyl Ezgü, F, Tokatli, A, Czartoryska, B, Bosschaart, AN, van den Bos-Terpstra, F, Puissant, H, Bürger, F, Omran, H, Eckert, D, Filocamo, M, Simeonov, E, Willems, PJ, Wevers, RA, Niermeijer, MF, Halley, DJ, Poorthuis, BJ, van Diggelen, OP

Hum Mutat 2010
3196333 Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase

Robertson, DA, Freeman, C, Nelson, PV, Morris, CP, Hopwood, JJ

Biochem Biophys Res Commun 1988
Participants
Input
Output
Participant Of
hasEvent
Catalyst Activity
Catalyst Activity
Title
N-acetylglucosamine-6-sulfatase activity of OxA-GNS [lysosomal lumen]
Physical Entity
OxA-GNS [lysosomal lumen]
Activity
N-acetylglucosamine-6-sulfatase activity (0008449)
Orthologous Events
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Bos taurus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Canis familiaris)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Danio rerio)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Drosophila melanogaster)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Gallus gallus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Mus musculus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Rattus norvegicus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Sus scrofa)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Xenopus tropicalis)
Authored
Jassal, B  (2011-10-05)
Reviewed
D'Eustachio, P  (2012-03-28)
Created
Jassal, B  (2011-10-06)
Cite Us!