Mouse N-Sulfoglucosamine Sulfohydrolase (SGSH) ELISA Kit-Sandwich

In mice, the function of N-sulfoglucosamine sulfohydrolase (SGSH) is primarily associated with the lysosomal storage disease known as mucopolysaccharidosis type III A (MPS IIIA), or Sanfilippo syndrome type A. SGSH's main role is to remove the sulfate group from the non-reducing end glucosamine residue in heparan sulfate (HS) molecules. This process is crucial for the normal degradation of HS, as the accumulation of sulfated HS fragments can lead to lysosomal dysfunction and subsequently trigger neurodegenerative diseases. In mouse models of MPS IIIA, the absence of SGSH enzyme activity hinders HS degradation, leading to the buildup of degradation products like HS-NRE in the central nervous system and other organs, resulting in severe neuropathological changes.