N-Sulfoglucosamine Sulfohydrolase (SGSH)
HSS; MPS3A; SFMD; Sulfamidase
 HGNC 10818
 MGI 1350341
 PubMed 18407553
 UniProt P51688
 USCN H178
 Wiki SGSH
N-Sulfoglucosamine Sulfohydrolase (SGSH)
A number sign is used with this entry because the phenotype is caused by mutation in the gene encoding N-sulfoglucosamine sulfohydrolase (SGSH). The Sanfilippo syndrome, or mucopolysaccharidosis III, is a lysosomal storage disease due to impaired degradation of heparan sulfate.
The Sanfilippo syndrome is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. Type A has been reported to be the most severe, with earlier onset and rapid progression of symptoms and shorter survival
Organism species: Homo sapiens (Human)
CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPH178Hu01 Recombinant N-Sulfoglucosamine Sulfohydrolase (SGSH) SDS-PAGE; WB; ELISA; IP.
Antibodies MAH178Hu21 Monoclonal Antibody to N-Sulfoglucosamine Sulfohydrolase (SGSH) WB, ICC, IHC-P, IHC-F, ELISA
PAH178Hu01 Polyclonal Antibody to N-Sulfoglucosamine Sulfohydrolase (SGSH) WB, ICC, IHC-P, IHC-F, ELISA
Assay Kits SEH178Hu ELISA Kit for N-Sulfoglucosamine Sulfohydrolase (SGSH) Enzyme-linked immunosorbent assay
Organism species: Mus musculus (Mouse)
CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPH178Mu01 Recombinant N-Sulfoglucosamine Sulfohydrolase (SGSH) SDS-PAGE; WB; ELISA; IP.
RPH178Mu02 Recombinant N-Sulfoglucosamine Sulfohydrolase (SGSH) SDS-PAGE; WB; ELISA; IP.
Antibodies PAH178Mu01 Polyclonal Antibody to N-Sulfoglucosamine Sulfohydrolase (SGSH) IWB, ICC, IHC-P, IHC-F, ELISA
PAH178Mu02 Polyclonal Antibody to N-Sulfoglucosamine Sulfohydrolase (SGSH) IWB, ICC, IHC-P, IHC-F, ELISA
Assay Kits SEH178Mu ELISA Kit for N-Sulfoglucosamine Sulfohydrolase (SGSH) Enzyme-linked immunosorbent assay