13C6-GlcNS-α(1→4)-GlcUA ("13C6-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 13C6-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 13C6-GlcNS-GlcUA, and these additional components have no effect on the ability to quantify the 13C6-GlcNS-GlcUA.

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

13C6-GlcNS-GlcUA (Endogenous GAG NRE)

SKU: BM12B

13C6-GlcNS-GlcUA

Catalog Number: BM12B

Chemical Formula (mono-anion): C6 13C6 H20 N O14 S

Molar Mass (mono-anion): 440.30 g/mol

Approximate Amount per Assay: 1 pmol (DBS)
Herbst, Z.M., Hong, X., Urdaneta, L., Klein, T., Waggoner, C., Liao, H.C., Kubaski, F., Giugliani, R., Fuller, M. and Gelb, M.H., 2023. Endogenous, non-reducing end glycosaminoglycan biomarkers are superior to internal disaccharide glycosaminoglycan biomarkers for newborn screening of mucopolysaccharidoses and GM1 gangliosidosis. Molecular Genetics and Metabolism, 140(1-2), p.107632. DOI: 10.1016/j.ymgme.2023.107632

13C6-GlcNS-GlcUA (Endogenous GAG NRE)

PRODUCT INFO

LITERATURE

MSDS