3.10.1.1: N-sulfoglucosamine sulfohydrolase
This is an abbreviated version!
For detailed information about N-sulfoglucosamine sulfohydrolase, go to the full flat file.
Word Map on EC 3.10.1.1
-
3.10.1.1
-
mucopolysaccharidosis
-
lysosomal
-
sanfilippo
-
mps-iiia
-
naglu
-
n-sulfate
-
sulfate-derived
-
heparinum
-
medicine
-
oestrone
-
hgsnat
-
sumf1
-
iduronate
-
sulfatases
-
ubiquitin-positive
-
drug development
-
pharmacology
-
diagnostics
- 3.10.1.1
- mucopolysaccharidosis
- lysosomal
-
sanfilippo
-
mps-iiia
- naglu
-
n-sulfate
-
sulfate-derived
- heparinum
- medicine
- oestrone
- hgsnat
- sumf1
-
iduronate
-
sulfatases
-
ubiquitin-positive
- drug development
- pharmacology
- diagnostics
Reaction
Synonyms
2-desoxy-2-sulphamido-D-glucose sulphamidase, 2-desoxy-D-glucoside-2-sulphamate sulphohydrolase, heparan N-sulfatase, heparan-N-sulfatase, heparan-N-sulphatase, heparin sulfamidase, N-sulfoglucosamine sulfohydrolase, N-sulphoglucosamine, SGSH, sulfamate sulfohydrolase, sulfamidase, sulfoglucosamine sulfamidase, sulphamate sulphohydrolase, sulphamidase, sulphohydrolase
ECTree
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Reference on EC 3.10.1.1 - N-sulfoglucosamine sulfohydrolase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Dietrich, C.P.; Silva, M.E.; Michelacci, Y.M.
Sequential degradation of heparin in Flavobacterium heparinum. Purification and properties of five enzymes involved in heparin degradation
J. Biol. Chem.
248
6408-6415
1973
Pedobacter heparinus
Dietrich, C.P.
Enzymic degradation of heparin. A sulphamidase and a sulphoesterase from Flavobacterium heparinum
Biochem. J.
111
91-95
1969
Pedobacter heparinus
Kresse, H.
Mucopolysaccharidosis III A (sanfilipo A disease): deficiency of a heparin sulfamidase in skin fibroblasts and leucocytes
Biochem. Biophys. Res. Commun.
54
1111-1118
1973
Homo sapiens, Mammalia
Friedman, Y.; Arsenis, C.
Studies on the heparin sulphamidase activity from rat spleen. Intracellular distribution and characterization of the enzyme
Biochem. J.
139
699-708
1974
Mammalia, Rattus norvegicus
Paschke, E.; Kresse, H.
Multiple forms of 2-deoxy-D-glucoside-2-sulphamate sulphohydrolase from human placenta
Biochem. J.
181
677-684
1978
Homo sapiens, Mammalia
Mahuran, D.; Clements, P.; Hopwood, J.J.
A rapid four column purification of 2-deoxy-D-glucoside-2-sulphamate sulphohydrolase from human liver
Biochim. Biophys. Acta
757
359-365
1983
Homo sapiens, Mammalia
Freeman, C.; Hopwood, J.J.
Human liver sulphamate sulphohydrolase. Determination of native protein and subunit Mr values and influence of substrate aglycone structure on catalytic properties
Biochem. J.
234
83-92
1986
Homo sapiens, Mammalia
Bruce, J.S.; McLean, M.W.; Long, W.F.; Williamson, F.B.
Flavobacterium heparinum sulphamidase for D-glucosamine sulphamate. Purification and characterisation
Eur. J. Biochem.
165
633-638
1987
Pedobacter heparinus
Bielicki, J.; Hopwood, J.J.; Melville, E.L.; Anson, D.S.
Recombinant human sulphamidase: expression, amplification, purification and characterization
Biochem. J.
329
145-150
1998
Homo sapiens
Perkins, K.J; Byers, S.; Yogalingam, G; Weber, B.; Hopwood, J.J.
Expression and characterization of wild typ and mutant recombinant human sulfamidase. Implications for sanfilippo (mucopolysaccharidosis IIIA) syndrome
J. Biol. Chem.
274
37193-37199
1999
Homo sapiens
Anson, D.S.; Bielicki, J.
Sulphamidase
Int. J. Biochem. Cell Biol.
31
363-367
1999
Homo sapiens
Gabrielli, O.; Coppa, G.V.; Bruni, S.; Villani, G.R.; Pontarelli, G.; di Natale, P.
An adult Sanfilippo type A patient with homozygous mutation R206P in the sulfamidase gene
Am. J. Med. Genet. A
133
85-89
2005
Homo sapiens
Muschol, N.; Storch, S.; Ballhausen, D.; Beesley, C.; Westermann, J.C.; Gal, A.; Ullrich, K.; Hopwood, J.J.; Winchester, B.; Braulke, T.
Transport, enzymatic activity, and stability of mutant sulfamidase (SGSH) identified in patients with mucopolysaccharidosis type III A
Hum. Mutat.
23
559-566
2004
Homo sapiens (P51688), Homo sapiens
Savas, P.S.; Hemsley, K.M.; Hopwood, J.J.
Intracerebral injection of sulfamidase delays neuropathology in murine MPS-IIIA
Mol. Genet. Metab.
82
273-285
2004
Homo sapiens
Gliddon, B.L.; Yogalingam, G.; Hopwood, J.J.
Purification and characterization of recombinant murine sulfamidase
Mol. Genet. Metab.
83
239-245
2004
Mus musculus
Montfort, M.; Garrido, E.; Hopwood, J.J.; Grinberg, D.; Chabas, A.; Vilageliu, L.
Expression and functional characterization of human mutant sulfamidase in insect cells
Mol. Genet. Metab.
83
246-251
2004
Homo sapiens
King, B.; Savas, P.; Fuller, M.; Hopwood, J.; Hemsley, K.
Validation of a heparan sulfate-derived disaccharide as a marker of accumulation in murine mucopolysaccharidosis type IIIA
Mol. Genet. Metab.
87
107-112
2006
Mus musculus
Gliddon, B.L.; Hopwood, J.J.
Enzyme-replacement therapy from birth delays the development of behavior and learning problems in mucopolysaccharidosis type IIIA mice
Pediatr. Res.
56
65-72
2004
Homo sapiens
Hemsley, K.M.; Beard, H.; King, B.M.; Hopwood, J.J.
Effect of high dose, repeated intra-CSF injection of sulphamidase on neuropathology in MPS IIIA mice
Genes Brain Behav.
7
740-753
2008
Homo sapiens
Fraldi, A.; Hemsley, K.; Crawley, A.; Lombardi, A.; Lau, A.; Sutherland, L.; Auricchio, A.; Ballabio, A.; Hopwood, J.J.
Functional correction of CNS lesions in an MPS-IIIA mouse model by intracerebral AAV-mediated delivery of sulfamidase and SUMF1 genes
Hum. Mol. Genet.
16
2693-2702
2007
Mus musculus
Meyer, A.; Kossow, K.; Gal, A.; Steglich, C.; Muehlhausen, C.; Ullrich, K.; Braulke, T.; Muschol, N.
The mutation p.Ser298Pro in the sulphamidase gene (SGSH) is associated with a slowly progressive clinical phenotype in mucopolysaccharidosis type IIIA (Sanfilippo A syndrome)
Hum. Mutat.
29
770
2008
Homo sapiens
Hemsley, K.M.; King, B.; Hopwood, J.J.
Injection of recombinant human sulfamidase into the CSF via the cerebellomedullary cistern in MPS IIIA mice
Mol. Genet. Metab.
90
313-328
2007
Homo sapiens
Settembre, C.; Annunziata, I.; Spampanato, C.; Zarcone, D.; Cobellis, G.; Nusco, E.; Zito, E.; Tacchetti, C.; Cosma, M.P.; Ballabio, A.
Systemic inflammation and neurodegeneration in a mouse model of multiple sulfatase deficiency
Proc. Natl. Acad. Sci. USA
104
4506-4511
2007
Mus musculus
Sutherland, L.M.; Hemsley, K.M.; Hopwood, J.J.
Primary culture of neural cells isolated from the cerebellum of newborn and adult mucopolysaccharidosis type IIIA mice
Cell. Mol. Neurobiol.
28
949-959
2008
Mus musculus
Roberts, A.L.; Howarth, G.S.; Liaw, W.C.; Moretta, S.; Kritas, S.; Lymn, K.A.; Yazbeck, R.; Tran, C.; Fletcher, J.M.; Butler, R.N.; Byers, S.
Gastrointestinal pathology in a mouse model of mucopolysaccharidosis type IIIA
J. Cell. Physiol.
219
259-264
2009
Mus musculus
McIntyre, C.; Derrick Roberts, A.L.; Ranieri, E.; Clements, P.R.; Byers, S.; Anson, D.S.
Lentiviral-mediated gene therapy for murine mucopolysaccharidosis type IIIA
Mol. Genet. Metab.
93
411-418
2008
Mus musculus
Urayama, A.; Grubb, J.H.; Sly, W.S.; Banks, W.A.
Mannose 6-phosphate receptor-mediated transport of sulfamidase across the blood-brain barrier in the newborn mouse
Mol. Ther.
16
1261-1266
2008
Mus musculus, Homo sapiens (P51688)
Muschol, N.; Pohl, S.; Meyer, A.; Gal, A.; Ullrich, K.; Braulke, T.
Residual activity and proteasomal degradation of p.Ser298Pro sulfamidase identified in patients with a mild clinical phenotype of Sanfilippo A syndrome
Am. J. Med. Genet. A
155A
1634-1639
2011
Homo sapiens
Sidhu, N.S.; Schreiber, K.; Proepper, K.; Becker, S.; Uson, I.; Sheldrick, G.M.; Gaertner, J.; Kraetzner, R.; Steinfeld, R.
Structure of sulfamidase provides insight into the molecular pathology of mucopolysaccharidosis IIIA
Acta Crystallogr. Sect. D
70
1321-1335
2014
Homo sapiens (P51688)
Whyte, L.; Hopwood, J.; Hemsley, K.; Lau, A.
Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates
Mol. Genet. Metab. Rep.
5
60-62
2015
Homo sapiens, Mus musculus, Mus musculus C57BL/6
Maccari, F.; Sorrentino, N.C.; Mantovani, V.; Galeotti, F.; Fraldi, A.; Volpi, N.
Glycosaminoglycan levels and structure in a mucopolysaccharidosis IIIA mice and the effect of a highly secreted sulfamidase engineered to cross the blood-brain barrier
Metab. Brain Dis.
32
203-210
2017
Homo sapiens (P51688), Homo sapiens
Jones, S.A.; Breen, C.; Heap, F.; Rust, S.; de Ruijter, J.; Tump, E.; Marchal, J.P.; Pan, L.; Qiu, Y.; Chung, J.K.; Nair, N.; Haslett, P.A.; Barbier, A.J.; Wijburg, F.A.
A phase 1/2 study of intrathecal heparan-N-sulfatase in patients with mucopolysaccharidosis IIIA
Mol. Genet. Metab.
118
198-205
2016
Homo sapiens (P51688), Homo sapiens
Wijburg, F.A.; Whitley, C.B.; Muenzer, J.; Gasperini, S.; Del Toro, M.; Muschol, N.; Cleary, M.; Sevin, C.; Shapiro, E.; Bhargava, P.; Kerr, D.; Alexanderian, D.
Intrathecal heparan-N-sulfatase in patients with Sanfilippo syndrome type A A phase IIb randomized trial
Mol. Genet. Metab.
126
121-130
2019
Homo sapiens (P51688), Homo sapiens
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