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首页> 外文期刊>CHROMATOGRAPHY >Quantification of Biological Thiols in the Plasma of a Homocystinuria Model with Cystathionine β-Synthase Deficiency Utilizing Hydrophilic Interaction Liquid Chromatography and Fluorescence Detection
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Quantification of Biological Thiols in the Plasma of a Homocystinuria Model with Cystathionine β-Synthase Deficiency Utilizing Hydrophilic Interaction Liquid Chromatography and Fluorescence Detection

机译：亲水相互作用液相色谱法和荧光检测法定量测定胱氨酸硫代β-合酶缺乏症同型半胱氨酸尿症模型血浆中的生物硫醇

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Biological thiols – homocysteine (Hcy), cysteine (Cys), γ -glutamylcysteine (γ GluCys), glutathione (GSH), and cysteinylglycine (CysGly) – in plasma samples of cystathionine β-synthase (CBS, EC 4.2.1.22)-deficient mice were quantified using hydrophilic interaction liquid chromatography (HILIC) and fluorescence detection. Mice deficient in CBS provide a model mouse of homocystinuria, an inherited metabolic disease characterized by the abnormal accumulation of Hcy in urine and blood. For quantification, the thiols were derivatized with ammonium 7-fluoro-2,1,3-benzoxadiazole-4-sulfonate (SBD-F), followed by separation with an amide column containing a mobile phase of acetonitrile–40 mM ammonium formate buffer (pH 3.0) (75/25, v/v). The total concentrations of Hcy in the plasma samples of CBS-wild type (-WT), -heterozygous (-Hetero), and -knockout (-KO) mice were 24.7, 29.3, and 237.7 μmol/L, respectively; 236.4, 178.7, and 78.5 μmol/L for Cys; 5.80, 4.87, and 0.75 μmol/L for γ GluCys; 64.6, 74.0, and 51.2 μmol/L for GSH; and 2.48, 3.98, and 0.90 μmol/L for CysGly. The concentration of Hcy was significantly increased, while Cys, γ GluCys, and CysGly concentrations were significantly decreased in CBS-KO mice compared to those in CBS-WT mice. The results indicated that biological thiols other than Hcy could also be utilized for the evaluation and investigation of homocystinuria pathologies.
机译：胱硫醚β-合酶（CBS，EC）血浆样品中的生物硫醇-高半胱氨酸（Hcy），半胱氨酸（Cys），γ-谷氨酰半胱氨酸（<γ> GluCys），谷胱甘肽（GSH）和半胱氨酸甘氨酸（CysGly）使用亲水相互作用色谱法（HILIC）和荧光检测对4.2.1.22）缺陷的小鼠进行定量。缺乏CBS的小鼠提供了高半胱氨酸尿症的模型小鼠，高半胱氨酸尿症是一种遗传性代谢疾病，其特征在于尿液和血液中Hcy的异常积累。为了定量，将硫醇用7-氟-2,1,3-苯并恶二唑-4-磺酸铵（SBD-F）衍生，然后用含有乙腈–40 mM甲酸铵缓冲液流动相的酰胺柱分离（ pH 3.0）（75/25，v / v）。 CBS野生型（-WT），-杂合子（-Hetero）和-knockout（-KO）小鼠血浆样品中Hcy的总浓度分别为24.7、29.3和237.7μmol/ L。 Cys为236.4、178.7和78.5μmol/ L; γGluCys为5.80、4.87和0.75μmol/ L; GSH为64.6、74.0和51.2μmol/ L; CysGly为2.48、3.98和0.90μmol/ L。与CBS-WT小鼠相比，CBS-KO小鼠中Hcy的浓度显着增加，而Cys，γGluCys和CysGly的浓度显着降低。结果表明，除Hcy外，其他生物硫醇也可用于高半胱氨酸尿症的病理学评估和研究。

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