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Browse our SGSH Proteins (SGSH)

Full name:
N-Sulfoglucosamine Sulfohydrolase Proteins (SGSH)
On are 7 N-Sulfoglucosamine Sulfohydrolase (SGSH) Proteins from 5 different suppliers available. Additionally we are shipping SGSH Antibodies (64) and SGSH Kits (8) and many more products for this protein. A total of 84 SGSH products are currently listed.
4632406A19Rik, HSS, MPS3A, SFMD
list all proteins Gene Name GeneID UniProt
SGSH 6448 P51688
Rat SGSH SGSH 688293  
Mouse SGSH SGSH 27029  

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SGSH Proteins (SGSH) by Origin

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Human N-Sulfoglucosamine Sulfohydrolase (SGSH) interaction partners

  1. results demonstrate that a single systemic scAAVrh74-hSGSH delivery mediated efficient restoration of SGSH activity and resulted in a near complete correction of MPS IIIA molecular pathology

  2. The crystal structure of glycosylated sulfamidase provides insight into the diverse effects of pathogenic mutations on sulfamidase function in mucopolysaccharidosis type IIIA.

  3. Pre-symptomatic treatment of progressive neurodegenerative disease (mucopolysaccharidosis type IIIA) via intra-cerebrospinal fluid injection of recombinant human SGSH mediates highly significant reductions in neuropathology in a canine model.

  4. Molecular genetics of mucopolysaccharidosis type IIIA and IIIB: Diagnostic, clinical, and biological implications

  5. Sanfilippo syndrome (subtypes A and B) in Turkey: identification of novel mutations in SGSH and NAGLU (show NAGLU Proteins)

  6. By assessing the degree of developmental regression over time a group of 7 pts (show PTS Proteins) with a slowly progressive course of MPSIIIA were identified. In these 7 pts (show PTS Proteins) and in 3 other mildly affected pts (show PTS Proteins) missense mutation c.892T>C (p.Ser298Pro) was found on 1 allele

SGSH Protein Profile

Protein Summary

This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.

Alternative names and synonyms associated with SGSH

  • N-sulfoglucosamine sulfohydrolase (SGSH)
  • N-sulfoglucosamine sulfohydrolase (Sgsh)
  • N-sulfoglucosamine sulfohydrolase (sulfamidase) (Sgsh)
  • 4632406A19Rik protein
  • HSS protein
  • MPS3A protein
  • SFMD protein

Protein level used designations for SGSH

N-sulphoglucosamine sulphohydrolase , heparan sulfate sulfatase , mucopolysaccharidosis type IIIA , sulfoglucosamine sulfamidase , sulphamidase , N-sulfoglucosamine sulfohydrolase

6448 Homo sapiens
688293 Rattus norvegicus
27029 Mus musculus
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