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通过LC-MS分解素酸盐导致了PGM1-CDG病理生理学的新见解

Karen Driesen^1,2,3, Sam De Craemer^1,2, Eva Morava⁴

¹Laboratory of Applied Mass Spectrometry, Department of Cellular and Molecular Medicine, KU Leuven, Leuven 3000, Belgium.

|September 29, 2025

概括

我们开发了两种液体色谱-MS方法来识别和量化人类的酸盐. 这些方法准确地测量细胞中的黑索酸盐概况,有助于研究代谢障碍,如先天性糖化 (CDG) 障碍.

科学领域:

代谢学代谢学代谢学
生物化学生物化学
分析化学分析化学

背景情况:

酸在新陈代谢途径中至关重要,如糖解和糖化.
了解赫索索-酸盐水平对于研究诸如先天性糖化失调 (CDG) 等疾病至关重要.
使用质谱法 (MS) 来区分结构异构体酸和酸盐是具有挑战性的,因为它们具有相似的物理化学性质.

研究的目的:

开发和比较优化的液体色谱-MS方法来识别人类的酸盐.
为了能够准确量化赫索索酸盐用于标记物代谢学应用.
在CDG和治疗反应的背景下分析赫索索-酸盐概况.

主要方法:

开发和比较两个优化的液态色谱-MS方法.
对线性 (3-4 个数量级) 和定量化极限 (0.5-50 nM) 的方法的验证.
应用方法来分析用银河糖治疗的糖突变酶1 (PGM1) -CDG纤维细胞中的黑色素酸盐概况.

主要成果:

成功识别和量化了8种赫索索单酸盐和2种赫索双酸盐.
方法显示高线性和灵敏度适合细胞度.
首份关于CDG纤维细胞中黑色酸盐概况及其在银河糖治疗后的变化的报告.

结论:

开发的LC-MS方法是有效的区分和量化人类的赫索索-酸盐在标记物代谢学.
这些方法为CDG和其他代谢障碍的分子基础提供了宝贵的见解.
这项研究证明了这些方法在分析疾病特异性代谢变化和治疗效果方面的有用性.

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