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Structure Characterization of a Novel Heparan Sulfate-Derived Saccharide Found in the Murine Model of Mucopolysaccharidosis Type I

机译:在粘性多糖分子中鼠霉素模型中发现的新型硫酸乙酰肝素衍生糖的结构表征

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The unknown saccharide was determined as AMAC-labeled saturated disaccharide, IdoA-GlcNS6S-AMAC, which represents the terminal disaccharide at the non-reducing end of the HS chain. This structure was further verified using exact mass measurement and laronidase treatment. This saccharide uniquely increased in Idua~(-/-) mice, which demonstrates the HS degradation is blocked, and to some extent, explains the accumulation of GAGs in MPSIH disease.
机译:未知的糖化为Amac标记的饱和二糖,IDOA-GLCNS6S-AMAc,其代表HS链的非还原末端的末端二糖。使用精确的质量测量和拉隆酶治疗进一步验证该结构。这种糖类在IDUA〜( - / - )小鼠中唯一增加,其证明HS降解被阻断,并且在一定程度上解释了MPSIH疾病中的GAG的积累。

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