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N-sulphoglucosamine sulphohydrolase

Catalyzes a step in lysosomal heparan sulfate degradation.

Below are the list of possible N-sulphoglucosamine sulphohydrolase products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.

N-sulphoglucosamine sulphohydrolase

 N-sulphoglucosamine sulphohydrolase ELISA Kit
 N-sulphoglucosamine sulphohydrolase Recombinant
 N-sulphoglucosamine sulphohydrolase Antibody
Also known as N-sulphoglucosamine sulphohydrolase (Sulfoglucosamine sulfamidase) (Sulphamidase).
SGSH: Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A); also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 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. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. Belongs to the sulfatase family.

Protein type: EC; Glycan Metabolism - glycosaminoglycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: 17q25.3

Cellular Component: lysosomal lumen; lysosome

Molecular Function: N-sulfoglucosamine sulfohydrolase activity

Biological Process: glycosaminoglycan catabolic process; heparan sulfate proteoglycan catabolic process

Disease: Mucopolysaccharidosis, Type Iiia
 SGSH Recombinant
 SGSH Antibody
 HSS Recombinant
 HSS Antibody
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