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