top of page

GlcNS-α(1→4)-GlcUA ("GlcNS-GlcUA") is the Endogenous GAG NRE quantitative standard for the deficiency of N-sulfoglucosamine sulfohydrolase (SGSH), which results in accumulation of Heparan Sulfate. 

 

The absolute amount of GlcNS-GlcUA in the vial was determined using quantitative NMR to have the stated value on the vial. The vial has additional components, but none are isobaric with GlcNS-GlcUA, and these additional components have no effect on the ability to quantify the GlcNS-GlcUA. 

 

Mucopolysaccharidosis type IIIA,  MPS-IIIA, MPS-3A, Sanfilippo Syndrome A

 

GlcNS-GlcUA (Endogenous GAG NRE)

SKU: BM12A
  • GlcNS-GlcUA

    Catalog Number: BM12A

     

    Chemical Formula (mono-anion):                               C12 H20 N O14 S      

    Molar Mass (mono-anion): 434.35 g/mol

bottom of page