Information on EC 3.1.6.14 - N-acetylglucosamine-6-sulfatase

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The expected taxonomic range for this enzyme is: Eukaryota, Bacteria

EC NUMBER
COMMENTARY
3.1.6.14
-
RECOMMENDED NAME
GeneOntology No.
N-acetylglucosamine-6-sulfatase
REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
hydrolysis of the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of heparan sulfate and keratan sulfate
show the reaction diagram
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
hydrolysis of sulfuric ester
-
-
-
-
PATHWAY
KEGG Link
MetaCyc Link
Glycosaminoglycan degradation
-
Metabolic 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.
SYNONYMS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
2-acetamido-2-deoxy-D-glucose 6-sulfate sulfatase
-
-
-
-
6-O-sulfatase
-
-
acetylglucosamine 6-sulfatase
-
-
-
-
chondroitinsulfatase
-
-
-
-
G6S
-
-
-
-
glucosamine 6-O-sulfatase
-
-
-
-
glucosamine 6-O-sulfatase
-
-
glucosamine-6-sulfatase
-
-
-
-
glucosamine-6-sulfate sulfatase
-
-
-
-
heparin/heparan sulfate 6-O-sulfatase
-
-
HSGAG sulfatase
-
-
N-acetylglucosamine 6-sulfate sulfatase
-
-
-
-
N-acetylglucosamine-6-sulfatase
P15586
-
O,N-disulfid O-sulfohydrolase
-
-
-
-
sulfatase, acetylglucosamine 6-
-
-
-
-
CAS REGISTRY NUMBER
COMMENTARY
60320-99-2
-
ORGANISM
COMMENTARY
LITERATURE
SEQUENCE CODE
SEQUENCE DB
SOURCE
Nubian goat, caprine
-
-
Manually annotated by BRENDA team
2 major forms: A, B , 2 minor form: C and D
-
-
Manually annotated by BRENDA team
patient with a single base pair deletion which causes a frameshift and premature termination of the protein
-
-
Manually annotated by BRENDA team
precursor
P15586
UniProt
Manually annotated by BRENDA team
strain RS2
-
-
Manually annotated by BRENDA team
strain RS2
-
-
Manually annotated by BRENDA team
Tagelus gibbus
mollusc
-
-
Manually annotated by BRENDA team
SUBSTRATE
PRODUCT                      
REACTION DIAGRAM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
(Substrate)
LITERATURE
(Substrate)
COMMENTARY
(Product)
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
4-methylumbelliferyl N-acetyl-beta-D-glucosamine 6-sulfate + H2O
4-methylumbelliferyl N-acetyl-beta-D-glucosamine + sulfate
show the reaction diagram
-
-
-
-
?
4-methylumbelliferyl sulfate + H2O
4-methylumbelliferone + sulfate
show the reaction diagram
-
fluorogenic substrate
-
-
?
alpha-D-glucosamine N2,O6-bissulfate + H2O
?
show the reaction diagram
-
-
-
-
?
glucose 6-sulfate + H2O
glucose + sulfate
show the reaction diagram
-
-
-
-
?
glucose 6-sulfate + H2O
glucose + sulfate
show the reaction diagram
-
no activity detected in cultured skin fibroblasts
-
-
-
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 + H2O
N-acetyl-D-glucosamine + sulfate
show the reaction diagram
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate + H2O
N-acetyl-D-glucosamine + sulfate
show the reaction diagram
-
lower activity in cultured skin fibroblasts compared to trisaccharides
-
-
?
N-acetyl-D-glucosamine 6-sulfate + H2O
N-acetyl-D-glucosamine + sulfate
show the reaction diagram
-
higher activity in liver compared to trisaccharides
-
-
?
N-acetyl-D-glucosamine 6-sulfate + H2O
N-acetyl-D-glucosamine + sulfate
show the reaction diagram
-
in heparan
-
?
N-acetyl-D-glucosamine 6-sulfate + H2O
N-acetyl-D-glucosamine + sulfate
show the reaction diagram
-
in heparan
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
Tagelus gibbus
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
catalyzes hydrolysis of sulfate from internal N-acetylglucosamine 6-sulfate at a lower rate
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
in substrates bearing a N-acetylglucosamine 6-sulfate residue at its nonreducing terminus derived from heparan sulfate and keratan sulfate
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
Tagelus gibbus
-
acts on polymer chain prior to depolymerization
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
acts also as endosulfatase
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
specific exosulfatase for desulfation of the nonreducing terminal N-acetylglucosamine 6-sulfate residues of heparansulfate
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
different enzymatic activities towards oligosaccharides derived from keratan sulfate or heparan sulfate
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
has no activity towards heparin, heparansulfate and keratansulfate, increase of activity in monosulfated tetra- or trisaccharides, maximum activity in sulfated monosaccharides
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
non-arylsulfatase
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
in oligo-and monosaccharides derived from heparan sulfate and keratan sulfate
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
different enzymatic hydrolysis for heparan sulfate and keratan sulfate
-
-
?
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-idonic acid + 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)-(1-3)-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-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
-
N-sulfated and N-acetylated derivates of
-
-
?
O-(alpha-glucosamine 6-sulfate)-(1-4)-O-uronosyl-(1-4)-2.5-anhydro-D-mannitol + H2O
O-(alpha-glucosamine)-(1-4)-O-uronosyl-(1-4)-2.5-anhydro-D-mannitol + 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)-(1-3)-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
-
30 fold higher activity in cultured skin fibroblasts compared to di- and monosaccharides
-
-
?
trisaccharide derived from heparansulfate with a N-acetylglucosamine 6-sulfate residue + H2O
trisaccharide with a N-acetylglucosamine + sulfate
show the reaction diagram
-
lower activity in liver compared to monosaccharides
-
-
?
glucose 6-sulfate + H2O
glucose + sulfate
show the reaction diagram
-
about 55% of the activity with N-acetyl-D-glucosamine 6-sulfate
-
-
?
additional information
?
-
-
involved in catabolism of heparan sulfate
-
?
additional information
?
-
-
the enzyme shows O-desulphation activity towards heparan sulphate-derived saccharides. No activity with glucose 3-sulfate, galactose 4-sulfate, galactose 6-sulfate and N-acetylgalactosamine 4-sulfate
-
-
-
additional information
?
-
P15586
involved in degradation of heparan sulfate, causing Mucopolysaccharidosis type IIID
-
-
-
additional information
?
-
-
heparin/heparan sulfate 6-O-sulfatase is a predominantly exolytic enzyme that specifically acts on N-sulfated or N-acetylated 6-O-sulfated glucosamines present at the non-reducing end of heparin and heparan sulfate glycosaminoglycan oligosaccharide substrates
-
-
-
NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
(Substrate)
LITERATURE
(Substrate)
COMMENTARY
(Product)
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
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
Tagelus gibbus
-
-
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
in oligo-and monosaccharides derived from heparan sulfate and keratan sulfate
-
-
?
N-acetyl-D-glucosamine 6-sulfate units + H2O
N-acetyl-D-glucosamine units + sulfate
show the reaction diagram
-
different enzymatic hydrolysis for heparan sulfate and keratan sulfate
-
-
?
additional information
?
-
-
involved in catabolism of heparan sulfate
-
?
METALS and IONS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
Ca2+
-
stimulated 2-3fold by 5 mM Ca2+
INHIBITORS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
Bovine serum albumin
-
-
-
Bovine serum albumin
-
inhibits only the purified enzyme
-
EDTA
-
less than 20% activity in the presence of 1 mM EDTA
NaCl
-
inhibits at 0.15 M
ACTIVATING COMPOUND
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
Bovine serum albumin
-
stimulates only in urine at pH 3.9
-
dithiothreitol
-
maximum activity at 0.1 mM
dithiothreitol
-
stimulates only the purified enzyme
KM VALUE [mM]
KM VALUE [mM] Maximum
SUBSTRATE
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
5.8
-
4-Methylumbelliferyl sulfate
-
-
0.062
-
glucose 6-sulfate
-
form A and B
7.7
-
glucose 6-sulfate
-
-
4.2
-
N-acetyl-D-glucosamine 6-sulfate
-
-
13
-
N-acetyl-D-glucosamine 6-sulfate
-
pH 7.0, 37C
0.0071
-
N-acetylglucosamine 6-sulfate
-
-
0.33
-
N-acetylglucosamine 6-sulfate
-
-
0.0108
-
O-(alpha-2-sulfamino glucosamine 6-sulfate)-(1-4)-L-idose
-
-
0.0111
-
O-(alpha-glucosamine 6-sulfate)-(1-3)-L-idonic acid
-
form C
0.0117
-
O-(alpha-glucosamine 6-sulfate)-(1-3)-L-idonic acid
-
form A
0.0142
-
O-(alpha-glucosamine 6-sulfate)-(1-3)-L-idonic acid
-
form B
0.0482
-
O-(alpha-glucosamine 6-sulfate)-(1-3)-L-idonic acid
-
-
0.0005
0.004
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 urine depending on pH and presence of bovine serum albumin
0.0035
-
O-(alpha-glucosamine 6-sulfate)-(1-4)-L-O-(alpha-iduronic acid 2-sulfate)-(1-4)-D-O-2,5-anhydro mannitol 6-sulfate
-
-
0.009
0.017
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
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
-
in leucocytes
0.0072
-
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-3)-L-idonic acid
-
in fibroblasts
0.0153
-
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-3)-L-idonic acid
-
in leucocytes
0.027
-
O-(alpha-N-acetyl glucosamine 6-sulfate)-(1-3)-L-idonic acid
-
in urine
0.15
-
trisaccharide with a N-acetylglucosamine 6-sulfate residue
-
-
-
IC50 VALUE [mM]
IC50 VALUE [mM] Maximum
INHIBITOR
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
SPECIFIC ACTIVITY [µmol/min/mg]
SPECIFIC ACTIVITY MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
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
0.00044
-
-
cell extract after growth on galactose
0.00086
-
-
cell extract after growth on mucin
0.0026
-
-
form C
0.23
-
-
form A
0.254
-
-
form B
0.922
-
-
-
pH OPTIMUM
pH MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
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
6
-
depending on substrate structure, overview
4.2
-
-
in fibroblasts
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
5.2
-
-
-
5.5
-
-
trisaccharide
5.7
-
-
glucose 6-sulfate, N-acetylglucosamine 6-sulfate
6.5
-
-
N-acetylglucosamine 6-sulfate
6.5
-
-
-
pH RANGE
pH RANGE MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
4
9
-
-
TEMPERATURE OPTIMUM
TEMPERATURE OPTIMUM MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
37
-
-
-
pI VALUE
pI VALUE MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
8.6
-
-
calculated from amino acid sequence
LOCALIZATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
-
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
MOLECULAR WEIGHT
MOLECULAR WEIGHT MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
10200
-
-
of the recombinant protein
52500
-
-
SDS-PAGE, reduced
57700
-
-
form A, form B, amino acid analysis
57800
-
-
SDS-PAGE, nonreduced
60200
-
-
gel filtration
61570
-
-
calculated from amino acid sequence
75000
-
-
form B, gel filtration
75000
-
-
-
75000
-
-
gel filtration
78000
-
-
form A, gel filtration
78000
-
-
-
78000
-
-
6 individual activites determined by chromatofocusing
94000
-
-
form C, gel filtration
94000
-
-
reduced, SDS-PAGE
97000
-
-
gel filtration
100000
-
-
chromatography, SDS-PAGE; heterogenous N-terminus
119000
-
-
SDS-PAGE
SUBUNITS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
dimer
-
1 * 48000, 1 * 32000, form B, SDS-PAGE
dimer
-
-
monomer
-
1 * 78000, form A, SDS-PAGE
monomer
-
-
monomer
-
1 * 75000, SDS-PAGE under reducing and non-reducing conditions
trimer
-
1 * 48000, 1 * 45000, 1 * 32000, form C, SDS-PAGE
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
glycoprotein
-
-
glycoprotein
-
20 sites of N-glycosylation
glycoprotein
-
mannose-6-phosphorylation, carbohydrate modification for lysosomal enzymes; treatment with PNGase
pH STABILITY
pH STABILITY MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
4
9
-
no activity below pH 4 or above pH 9
4.5
-
-
24 h, complete inactivation below pH 4.5
9.5
-
-
24 h, complete inactivation above pH 9.5
TEMPERATURE STABILITY
TEMPERATURE STABILITY MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
15
-
-
no activity
60
-
-
no activity
70
-
-
after 3 min 70% loss of activity
GENERAL STABILITY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
unstable to freezing, thawing
-
bovine serum albumin stabilizes
-
the enzyme shows higher activity in acetate buffer when compared with sulfonate buffers such as MES and MOPS
-
STORAGE STABILITY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
-80C, pH 5.5, 0.02% NaN3, several months, no loss of activity
-
4C, pH 5.5, 0.02% NaN3, 1 month
-
4C, 2 months, pH 5.7, form A and B retain 95% of original activity
-
Purification/COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
to homogeneity
-
of the recombinant protein
-
6 individual activites determined
-
form A and B, form C: partial
-
partial
Tagelus gibbus
-
Cloned/COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
expression in CHO cells
-
expression in Escherichia coli
-
expressed in Escherichia coli BL21(DE3) cells
-
ENGINEERING
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
C80A
-
inactive
D186A
-
53% relative activity after 0.5 h and 92% relative activity after 5 h compared to the wild type enzyme
D374A
-
inactive
R102X
-
nonsense mutation
additional information
P15586
mutation of glutamine 390 to stop-codon causes Mucopolysaccharidosis type IIID
APPLICATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
medicine
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endocytosis of the recombinant enzyme by human fibroblasts
medicine
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normal activity in patient G.G.,cell line GM 2243
medicine
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heparan sulfate storage disorder not keratan storage disorder
medicine
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Sanfilippo D Syndrome: N-acetylglucosamine 6-sulfate sulfatase deficiency, mucopolysaccharidosis type IIID
medicine
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defect in enzyme function leads to mucopolysaccharidosis type IIID, i.e. Sanfilippo syndrome Type D