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. Author manuscript; available in PMC: 2021 Jul 7.
Published in final edited form as: Biochem J. 2020 Sep 18;477(17):3433–3451. doi: 10.1042/BCJ20200546

Figure 4. Arsk-deficient mice accumulate 2-O-sulfated non-reducing end (NRE) biomarkers.

Figure 4.

HS NREs derived from brain and kidney (A) and isolated liver lysosomes (B) of Arsk KO or WT mice were obtained by GAG-depolymerization using bacterial lyases. Quantification of HS NREs from tissues or liver lysosomes was achieved by GRIL-LC/MS with known amounts of [13C6]aniline-labeled internal standards including the specific G2S0 (A left panel; B) and G2A0 (A right panel) disaccharides. (n = 3 for each genotype, values represent mean ± SEM, *P < 0.05, **P < 0.01). (C) Kidney-derived 2-O-sulfat HS NREs were incubated with recombinant human (rh)ARSK (15 ng), rhIDS (8 ng) or with buffer overnight and quantified as described above. (D) Quantification of CS-derived NREs from brain and kidney of WT and Arsk KO mice.