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Information on EC 3.1.6.14 - N-acetylglucosamine-6-sulfatase and Organism(s) Homo sapiens and UniProt Accession P15586

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EC Tree
     3 Hydrolases
         3.1 Acting on ester bonds
             3.1.6 Sulfuric-ester hydrolases
                3.1.6.14 N-acetylglucosamine-6-sulfatase
IUBMB Comments
May be identical with EC 3.1.6.11 disulfoglucosamine-6-sulfatase.
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Select one or more organisms in this record: ?
This record set is specific for:
Homo sapiens
UNIPROT: P15586
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Word Map
The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Synonyms
sulf-1, n-acetylglucosamine-6-sulfatase, heparan sulfate 6-o-endosulfatase, glucosamine-6-sulfatase, n-acetylglucosamine 6-sulfate sulfatase, 6-o-sulfatase, hs 6-o-endosulfatase, 2-acetamido-2-deoxy-d-glucose 6-sulfate sulfatase, heparan sulfate 6-o-endosulfatase1, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
heparan sulfate 6-O-endosulfatase
-
heparan sulfate 6-O-endosulfatase1
-
HS 6-O-endosulfatase
-
N-acetylglucosamine-6-sulfatase
-
2-acetamido-2-deoxy-D-glucose 6-sulfate sulfatase
-
-
-
-
acetylglucosamine 6-sulfatase
-
-
-
-
chondroitinsulfatase
-
-
-
-
glucosamine 6-O-sulfatase
-
-
-
-
glucosamine-6-sulfatase
-
-
-
-
glucosamine-6-sulfate sulfatase
-
-
-
-
N-acetylglucosamine 6-sulfate sulfatase
-
-
-
-
NG6S
-
-
O,N-disulfid O-sulfohydrolase
-
-
-
-
sulfatase, acetylglucosamine 6-
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
hydrolysis of sulfuric ester
-
-
-
-
PATHWAY SOURCE
PATHWAYS
SYSTEMATIC NAME
IUBMB Comments
N-acetyl-D-glucosamine-6-sulfate 6-sulfohydrolase
May be identical with EC 3.1.6.11 disulfoglucosamine-6-sulfatase.
CAS REGISTRY NUMBER
COMMENTARY hide
60320-99-2
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
4-methylumbelliferyl sulfate + H2O
4-methylumbelliferone + sulfate
show the reaction diagram
fluorogenic substrate 4-MUS
-
-
?
beta-D-GlcNAc6S-(1->4)-beta-D-GlcA-(1->4)-2,5-anhydro-D-mannitol + H2O
beta-D-GlcNAc-(1->4)-beta-D-GlcA-(1->4)-2,5-anhydro-D-mannitol + sulfate
show the reaction diagram
-
-
-
-
?
glucose 6-sulfate + H2O
glucose + sulfate
show the reaction diagram
N-acetyl galactosamine 6-sulfate + H2O
N-acetyl galactosamine + sulfate
show the reaction diagram
-
very low activity in cultured skin fibroblasts
-
-
?
N-acetyl-D-glucosamine 6-sulfate + H2O
N-acetyl-D-glucosamine + sulfate
show the reaction diagram
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
O-(alpha-2-sulfamino glucosamine 6-sulfate)-(1-4)-L-idonic acid + H2O
O-(alpha-2-sulfamino glucosamine)-(1-4)-L-idose + sulfate
show the reaction diagram
-
-
-
-
?
O-(alpha-2-sulfamino glucosamine 6-sulfate)-(1-4)-L-idose + H2O
O-(alpha-2-sulfamino glucosamine)-(1-4)-L-idose + sulfate
show the reaction diagram
-
-
-
-
?
O-(alpha-glucosamine 6-sulfate)-(1-3)-L-idonic acid + H2O
O-(alpha-glucosamine)-(1-3)-L-idonic acid + sulfate
show the reaction diagram
-
-
-
-
?
O-(alpha-glucosamine 6-sulfate)-(1-4)-L-O-(alpha-iduronic acid 2-sulfate)-(1-4)-D-O-2,5-anhydro mannitol 6-sulfate + H2O
O-(alpha-glucosamine)-(1-4)-L-O-(alpha-iduronic acid 2-sulfate)-(1-4)-D-O-2,5-anhydro mannitol 6-sulfate + sulfate
show the reaction diagram
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-3)-L-idonic acid + H2O
O-(alpha-N-acetyl glucosamine)-(1-3)-L-idonic acid + sulfate
show the reaction diagram
-
-
-
-
?
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-4)-L-idose-2-sulfate + H2O
O-(alpha-N-acetyl glucosamine)-(1-4)-L-idose-2-sulfate + sulfate
show the reaction diagram
-
same activity in cultured skin fibroblasts compared to monosaccharides
-
-
?
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-4)-L-iduronic acid-2-sulfate-(1-4)-2,5-anhydro-D-mannitol-6-sulfate + H2O
O-(alpha-N-acetyl glucosamine)-(1-4)-L-iduronic acid-2-sulfate-(1-4)-2,5-anhydro-D-mannitol-6-sulfate + sulfate
show the reaction diagram
-
30 fold higher activity in cultured skin fibroblasts compared to di- and monosaccharides
-
-
?
O-(beta-N-acetyl glucosamine 6-sulfate)-(1-3)-D-galactitol + H2O
O-(alpha-N-acetyl glucosamine)-(1-3)-D-galactitol + sulfate
show the reaction diagram
-
activity 100-fold less
-
-
?
trisaccharide derived from heparansulfate with a N-acetylglucosamine 6-sulfate residue + H2O
trisaccharide with a N-acetylglucosamine + sulfate
show the reaction diagram
trisaccharide with an N-acetylglucosamine 6-sulfate residue + H2O
trisaccharide with an N-acetylglucosamine + sulfate
show the reaction diagram
-
-
-
-
?
additional information
?
-
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
additional information
?
-
-
involved in catabolism of heparan sulfate
-
?
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Mg2+
required
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
bovine serum albumin
-
Hg2+
-
-
S2O32-
-
-
SO32-
-
-
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
bovine serum albumin
-
stimulates only in urine at pH 3.9
-
dithiothreitol
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.062 - 7.7
glucose 6-sulfate
13
N-acetyl-D-glucosamine 6-sulfate
-
pH 7.0, 37°C
0.0071 - 0.33
N-acetylglucosamine 6-sulfate
0.0108
O-(alpha-2-sulfamino glucosamine 6-sulfate)-(1-4)-L-idose
-
-
0.0111 - 0.0142
O-(alpha-glucosamine 6-sulfate)-(1-3)-L-idonic acid
0.0005 - 0.028
O-(alpha-glucosamine 6-sulfate)-(1-4)-L-O-(alpha-iduronic acid 2-sulfate)-(1-4)-D-O-2,5-anhydro mannitol 6-sulfate
0.0072 - 0.027
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-3)-L-idonic acid
0.15
trisaccharide with an N-acetylglucosamine 6-sulfate residue
-
-
-
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
0.00000006
-
N-acetyl galactosamine 6-sulfate of cultured skin fibroblasts
0.00000052
-
N-acetyl-D-glucosamine 6-sulfate of cultured skin fibroblasts
0.000001
-
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-4)-L-idose-2-sulfate of cultured skin fibroblasts
0.00003
-
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-4)-L-iduronic acid-2-sulfate-(1-4)-2,5-anhydro-D-6-sulfate of cultured skin fibroblasts
0.0026
-
form C
0.23
-
form A
0.254
-
form B
0.922
-
-
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7.5
assay at
3.9
-
O-(alpha-glucosamine 6-sulfate)-(1-4)-L-O-(alpha-iduronic acid 2-sulfate)-(1-4)-D-O-2,5-anhydro mannitol 6-sulfate in fibroblasts
4.1
-
O-(alpha-glucosamine 6-sulfate)-(1-4)-L-O-(alpha-iduronic acid 2-sulfate)-(1-4)-D-O-2,5-anhydro mannitol 6-sulfate in leucocytes
4.2
-
in fibroblasts
4.2 - 6
-
depending on substrate structure, overview
4.3
-
O-(alpha-glucosamine 6-sulfate)-(1-4)-L-O-(alpha-iduronic acid 2-sulfate)-(1-4)-D-O-2,5-anhydro mannitol 6-sulfate
4.5
-
in leukocytes
4.8
-
O-(alpha-2-sulfamino glucosamine 6-sulfate)-(1-4)-L-idose
5
-
forms: A, B, C
5.1
-
in urine
5.5
-
trisaccharide
5.7
-
glucose 6-sulfate, N-acetylglucosamine 6-sulfate
6.5
-
N-acetylglucosamine 6-sulfate
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
37
assay at
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
the heterodimeric enzyme is released in extracellular environment and/or retained at the cell membrane, probably by interaction with cell surface heparan sulfate
-
Manually annotated by BRENDA team
the heterodimeric enzyme is released in extracellular environment and/or retained at the cell membrane, probably by interaction with cell surface heparan sulfate
-
Manually annotated by BRENDA team
-
26% in fractions of secondary granules, 63% in fractions of primary granules
-
Manually annotated by BRENDA team
-
26% in fractions of secondary granules, 63% in fractions of primary granules
-
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
GNS_HUMAN
552
1
62082
Swiss-Prot
Mitochondrion (Reliability: 4)
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
100000
32000
45000
-
1 * 48000, 1 * 45000, 1 * 32000, form C, SDS-PAGE
48000
57700
-
form A, form B, amino acid analysis
75000
78000
94000
-
form C, gel filtration
97000
-
gel filtration
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
dimer
monomer
trimer
-
1 * 48000, 1 * 45000, 1 * 32000, form C, SDS-PAGE
additional information
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
proteolytic modification
Sulf-1 is synthesized as a about 125-kDa precursor that is cleaved by a furin-type proteinase. This process results in the formation of an active heterodimer, in which 75-kDa and 50-kDa subunits are linked by disulfide bonds
glycoprotein
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
pH STABILITY
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
4.5
-
24 h, complete inactivation below pH 4.5
135579
5 - 7.5
-
-
135579, 135581
9.5
-
24 h, complete inactivation above pH 9.5
135579
TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
70
-
after 3 min 70% loss of activity
GENERAL STABILITY
ORGANISM
UNIPROT
LITERATURE
bovine serum albumin stabilizes
-
STORAGE STABILITY
ORGANISM
UNIPROT
LITERATURE
4°C, 2 months, pH 5.7, form A and B retain 95% of original activity
-
PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
6 individual activites determined
-
form A and B, form C: partial
-
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
gene GNS, quantitative real-time PCR enzyme expression analysis
expression in Escherichia coli
-
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
tumour-necrosis factor-alpha (TNF-alpha) induces heparan sulfate 6-O-endosulfatase1 (Sulf-1) expression in fibroblasts via a mechanism involving NF-kappaB, ERK1/2, and p38 MAPK. Cell stimulation with TNF-alpha is accompanied by the secretion of an active form of Sulf-1. Regulation of Sulf-1 expression by TNF-alpha, overview
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Freeman, C.; Clements, P.R.; Hopwood, J.J.
Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization
Biochem. J.
246
347-354
1987
Homo sapiens
Manually annotated by BRENDA team
Freeman, C.; Hopwood, J.J.
Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties
Biochem. J.
246
355-365
1987
Bos taurus, Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Basner, R.; Kresse, H.; von Figura, K.
N-Acetylglucosamine-6-sulfate sulfatase from human urine
J. Biol. Chem.
254
1151-1158
1979
Homo sapiens
Manually annotated by BRENDA team
Elliott, H.; Hopwood, J.J.
Detection of the Sanfilippo D syndrome by the use of a radiolabeled monosaccharide sulfate as the substrate for the estimation of N-acetylglucosamine-6-sulfate sulfatase
Anal. Biochem.
138
205-209
1984
Homo sapiens
Manually annotated by BRENDA team
Di Ferrante, N.
N-acetylglucosamine-6-sulfate sulfatase deficiency reconsidered
Science
210
448
1980
Homo sapiens
Manually annotated by BRENDA team
Kresse, H.; Fuchs, W.; Glossl, J.; Holtfrerich, D.; Gilberg, W.
Liberation of N-acetylglucosamine-6-sulfate by human beta-N-acetylhexosaminidase A
J. Biol. Chem.
256
12926-12932
1981
Homo sapiens
Manually annotated by BRENDA team
Kresse, H.; von Figura, K.; Klein, U.; Glossl, J.; Paschke, E.; Pohlmann, R.
Enzymic diagnosis of the genetic mucopolysaccharide storage disorders
Methods Enzymol.
83
559-572
1982
Homo sapiens
Manually annotated by BRENDA team
Freeman, C.; Hopwood, J.J.
Sanfilippo D syndrome: estimation of N-acetylglucosamine-6-sulfatase activity with a radiolabeled monosulfated disaccharide substrate
Anal. Biochem.
176
244-248
1989
Homo sapiens
Manually annotated by BRENDA team
Kresse, H.; Paschke, E.; von Figura, K.; Gilberg, W.; Fuchs, W.
Sanfilippo disease type D: deficiency of N-acetylglucosamine-6-sulfate sulfatase required for heparan sulfate degradation
Proc. Natl. Acad. Sci. USA
77
6822-6826
1980
Homo sapiens
Manually annotated by BRENDA team
Robertson, D.A.; Freeman, C.; Morris, C.P.; Hopwood, J.J.
A cDNA clone for human glucosamine-6-sulphatase reveals differences between arylsulphatases and non-arylsulphatases
Biochem. J.
288
539-544
1992
Homo sapiens
Manually annotated by BRENDA team
Freeman, C.; Hopwood, J.J.
Human glucosamine-6-sulphatase deficiency. Diagnostic enzymology towards heparin-derived trisaccharide substrates
Biochem. J.
282
605-614
1992
Homo sapiens
Manually annotated by BRENDA team
Sleat, D.E.; Kraus, S.R.; Sohar, I.; Lackland, H.; Lobel, P.
alpha-Glucosidase and N-acetylglucosamine-6-sulphatase are the major mannose-6-phosphate glycoproteins in human urine
Biochem. J.
324
33-39
1997
Homo sapiens
Manually annotated by BRENDA team
Beesley, C.E.; Burke, D.; Jackson, M.; Vellodi, A.; Winchester, B.G.; Young, E.P.
Sanfilippo syndrome type D: identification of the first mutation in the N-acetylglucosamine-6-sulphatase gene
J. Med. Genet.
40
192-194
2003
Homo sapiens
Manually annotated by BRENDA team
Xu, S.; Zhao, L.; Larsson, A.; Smeds, E.; Kusche-Gullberg, M.; Venge, P.
Purification of a 75 kDa protein from the organelle matrix of human neutrophils and identification as N-acetylglucosamine-6-sulphatase
Biochem. J.
387
841-847
2005
Homo sapiens
Manually annotated by BRENDA team
Jansen, A.C.; Cao, H.; Kaplan, P.; Silver, K.; Leonard, G.; De Meirleir, L.; Lissens, W.; Liebaers, I.; Veilleux, M.; Andermann, F.; Hegele, R.A.; Andermann, E.
Sanfilippo syndrome type D: natural history and identification of 3 novel mutations in the GNS Gene
Arch. Neurol.
64
1629-1634
2007
Homo sapiens (P15586), Homo sapiens
Manually annotated by BRENDA team
Zhou, X.; Li, L.; Linhardt, R.J.; Liu, J.
Neutralizing the anticoagulant activity of ultra-low-molecular-weight heparins using N-acetylglucosamine 6-sulfatase
FEBS J.
280
2523-2532
2013
Homo sapiens
Manually annotated by BRENDA team
Sikora, A.-S.; Hellec, C.; Carpentier, M.; Martinez, P.; Delos, M.; Denys, A.; Allain, F.
Tumour-necrosis factor-alpha induces heparan sulfate 6-O-endosulfatase1 (Sulf-1) expression in fibroblasts
Int. J. Biochem. Cell Biol.
80
57-65
2016
Homo sapiens (P15586), Homo sapiens
Manually annotated by BRENDA team