N-acetylglucosamine-6-sulfatase

Gene ontology
Molecular function	sulfuric ester hydrolase activity protein binding catalytic activity hydrolase activity metal ion binding N-acetylglucosamine-6-sulfatase activity glycosaminoglycan binding
Cellular component	lysosome lysosomal lumen extracellular exosome extracellular region azurophil granule lumen ficolin-1-rich granule lumen
Biological process	metabolism glycosaminoglycan catabolic process keratan sulfate catabolic process glycosaminoglycan metabolic process neutrophil degranulation
Sources:Amigo / QuickGO

Orthologs

Species

Human

Mouse

Entrez


2799


75612

Ensembl


ENSG00000135677


ENSMUSG00000034707

UniProt


P15586


Q8BFR4

RefSeq (mRNA)


NM_002076


NM_029364 NM_001364695

RefSeq (protein)


NP_002067 NP_002067.1


NP_083640 NP_001351624

Location (UCSC)

Chr 12: 64.71 – 64.76 Mb

Chr 10: 121.2 – 121.23 Mb

PubMed search

^[3]

^[4]

Wikidata

View/Edit Human

View/Edit Mouse

N-acetylglucosamine-6-sulfatase (EC 3.1.6.14, glucosamine (N-acetyl)-6-sulfatase, systematic name N-acetyl-D-glucosamine-6-sulfate 6-sulfohydrolase) is an enzyme that in humans is encoded by the GNS gene.^[5] It is deficient in Sanfilippo Syndrome type IIId.^[6]^[7]^[8] It catalyses the 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.^[5]

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.^[5]

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

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000135677 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000034707 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ ^a ^b ^c "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. doi:10.1016/S0021-9258(17)34181-9. 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. doi:10.1016/S0021-9258(18)42985-7. 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 U.S. 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.

glycosaminoglycan anabolism

Heparan sulfate: EXT1; EXT2

Chondroitin sulfate: PAPSS1; PAPSS2

glycosaminoglycan catabolism

Hunter, Hurler	Iduronate-2-sulfatase Iduronidase
Sanfilippo, Sly	Heparan sulfamidase N-acetyltransferase Alpha-N-acetylglucosaminidase Glucuronidase N-acetylglucosamine-6-sulfatase
Morquio/Maroteaux-Lamy	Arylsulfatase B Galactosamine-6 sulfatase Beta-galactosidase (GLB1)

Hydrolase: esterases (EC 3.1)

3.1.1: Carboxylic
ester hydrolases

Lipase

Phospholipase
- A1
- A2
- B

3.1.2: Thioesterase

3.1.3: Phosphatase

3.1.4:
Phosphodiesterase

3.1.6: Sulfatase

Nuclease (includes
deoxyribonuclease
and ribonuclease)

3.1.11-16:
Exonuclease

Exodeoxyribonuclease	RecBCD
Exoribonuclease	Oligonucleotidase

3.1.21-31:
Endonuclease

Endodeoxyribonuclease	Deoxyribonuclease I Deoxyribonuclease II Deoxyribonuclease IV Restriction enzyme;see {{Restriction enzyme}} UvrABC endonuclease
Endoribonuclease	RNase III Drosha: Microprocessor complex Dicer: RNA-induced silencing complex RNase H 1 2A 2B 2C RNase P RNase A RNase Z RNase E 1 2 3 4/5 RNase T1
either deoxy- or ribo-	Nuclease S1 Mung bean nuclease Serratia marcescens nuclease Micrococcal nuclease

v t e Enzymes
Activity	Active site Binding site Catalytic triad Oxyanion hole Enzyme promiscuity Diffusion-limited enzyme Cofactor Enzyme catalysis
Regulation	Allosteric regulation Cooperativity Enzyme inhibitor Enzyme activator
Classification	EC number Enzyme superfamily Enzyme family List of enzymes
Kinetics	Enzyme kinetics Eadie–Hofstee diagram Hanes–Woolf plot Lineweaver–Burk plot Michaelis–Menten kinetics
Types	EC1 Oxidoreductases (list) EC2 Transferases (list) EC3 Hydrolases (list) EC4 Lyases (list) EC5 Isomerases (list) EC6 Ligases (list) EC7 Translocases (list)

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This page was last edited on 6 October 2023, at 20:36