Ulivelli Monica, Priora Raffaella, Di Giuseppe Danila, Coppo Lucia, Summa Domenico, Margaritis Antonios, Frosali Simona, Bartalini Sabina, Martini Giuseppe, Cerase Alfonso, Di Simplicio Paolo
Department of Medicine, Surgery, and Neuroscience, University of Siena, 53100, Siena, Italy.
Pharmacology Unit, Department of Neuroscience, University of Siena, 53100, Siena, Italy.
Amino Acids. 2016 Jun;48(6):1477-89. doi: 10.1007/s00726-016-2207-2. Epub 2016 Mar 11.
The toxicity risk of hyperhomocysteinemia is prevented through thiol drug administration which reduces plasma total homocysteine (tHcy) concentrations by activating thiol exchange reactions. Assuming that cysteine (Cys) is a homocysteinemia regulator, the hypothesis was verified in healthy and pathological individuals after the methionine loading test (MLT). The plasma variations of redox species of Cys, Hcy, cysteinylglycine, glutathione and albumin (reduced, HS-ALB, and at mixed disulfide, XSS-ALB) were compared in patients with cerebral small vessels disease (CSVD) (n = 11), multiple sclerosis (MS) (n = 12) and healthy controls (n = 11) at 2-4-6 h after MLT. In MLT-treated subjects, the activation of thiol exchange reactions provoked significant changes over time in redox species concentrations of Cys, Hcy, and albumin. Significant differences between controls and pathological groups were also observed. In non-methionine-treated subjects, total Cys concentrations, tHcy and thiol-protein mixed disulfides (CSS-ALB, HSS-ALB) of CSVD patients were higher than controls. After MLT, all groups displayed significant cystine (CSSC) increases and CSS-ALB decreases, that in pathological groups were significantly higher than controls. These data would confirm the Cys regulatory role on the homocysteinemia; they also explain that the Cys-Hcy mixed disulfide excretion is an important point of hyperhomocysteinemia control. Moreover, in all groups after MLT, significant increases in albumin concentrations, named total albumin (tALB) and measured as sum of HS-ALB (spectrophometric), and XSS-ALB (assayed at HPLC) were observed. tALB increases, more pronounced in healthy than in the pathological subjects, could indicate alterations of albumin equilibria between plasma and other extracellular spaces, whose toxicological consequences deserve further studies.
通过给予硫醇类药物可预防高同型半胱氨酸血症的毒性风险,这类药物通过激活硫醇交换反应来降低血浆总同型半胱氨酸(tHcy)浓度。假设半胱氨酸(Cys)是同型半胱氨酸血症的调节剂,该假设在蛋氨酸负荷试验(MLT)后,于健康个体和患病个体中得到验证。比较了脑小血管病(CSVD)患者(n = 11)、多发性硬化症(MS)患者(n = 12)和健康对照者(n = 11)在MLT后2 - 4 - 6小时时,Cys、Hcy、半胱氨酰甘氨酸、谷胱甘肽和白蛋白(还原型、HS - ALB以及混合二硫键型、XSS - ALB)的氧化还原物质的血浆变化情况。在接受MLT治疗的受试者中,硫醇交换反应的激活导致Cys、Hcy和白蛋白的氧化还原物质浓度随时间发生显著变化。在对照组和患病组之间也观察到了显著差异。在未接受蛋氨酸治疗的受试者中,CSVD患者的总Cys浓度、tHcy以及硫醇 - 蛋白质混合二硫键(CSS - ALB、HSS - ALB)高于对照组。MLT后,所有组的胱氨酸(CSSC)均显著增加,CSS - ALB均降低,患病组的变化显著高于对照组。这些数据将证实Cys对同型半胱氨酸血症的调节作用;它们还解释了Cys - Hcy混合二硫键的排泄是控制高同型半胱氨酸血症的一个重要环节。此外,在MLT后的所有组中,观察到白蛋白浓度显著增加,称为总白蛋白(tALB),通过分光光度法测定HS - ALB以及通过高效液相色谱法测定XSS - ALB的总和来衡量。tALB的增加在健康受试者中比在患病受试者中更为明显,这可能表明血浆与其他细胞外空间之间白蛋白平衡的改变,其毒理学后果值得进一步研究。