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首页> 外文期刊>Biochimica et biophysica acta. Molecular basis of disease: BBA >L-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload
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L-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload

机译：L-肉碱可防止在提交给赖氨酸过载的谷氨酸 - 辅酶脱氢酶缺陷小鼠中氧化胁迫

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The deficiency of the enzyme glutaryl-CoA dehydrogenase leads to predominant accumulation of glutaric acid (GA) in the organism and is known as glutaric acidemia type I (GA1). Despite the mechanisms of brain damage involved in GA1 are not fully understood, oxidative stress may be involved in this process. Treatment is based on protein/lysine (Lys) restriction and L-carnitine (L-car) supplementation. L-car was recently shown to have an important antioxidant role. A knockout mice model (Gcdh(-/-)) submitted to a dietary overload of Lys was developed to better understand the GA1 pathogenesis. In this study, we evaluated L-car and glutarylcarnitine levels, the lipid and protein damage, reactive oxygen species (ROS) production and antioxidant enzymes activities in striatum of Gcdh(-/-) and wild-type (WT) mice. We also determined the effect of the L-car treatment on these parameters. Thirty-day-old Gcdh(-/-) and WT mice were fed a normal chow (0.9% Lys) or submitted to a high Lys diet (4.7%) for 72 h. Additionally, these animals were administered with three intraperitoneal injections of saline or L-car in different times. Gcdh(-/-) mice were deficient in L-car and presented a higher glutarylcarnitine levels. They also presented lipid and protein damage, an increased ROS production and altered antioxidant enzymes compared to WT mice. Additionally, mice exposed to Lys overload presented higher alterations in these parameters than mice under normal diet, which were significantly decreased or normalized in those receiving L-car. Thus, we demonstrated a new beneficial effect of the L-car treatment attenuating or abolishing the oxidative stress process in Gcdh(-/-) mice.
机译：酶谷蛋白酶脱氢酶的缺陷导致生物体中戊二酸（Ga）的主要积累，并且称为戊二酸型I（Ga1）。尽管Ga1中涉及脑损伤的机制尚未完全明白，但可以参与该过程中的氧化应激。治疗基于蛋白质/赖氨酸（Lys）限制和L-肉碱（L-CAR）补充。最近显示L-Car具有重要的抗氧化作用。开发出提交给膳食过载的敲除小鼠模型（GCDH（ - / - ））以更好地理解GA1发病机制。在本研究中，我们评估了L-Car和谷蛋白水平，脂质和蛋白质损伤，脂质和蛋白质损伤，在GCH（ - / - ）和野生型（WT）小鼠的纹状体中的活性氧物质（ROS）产生和抗氧化酶活性。我们还确定了L-CAR处理对这些参数的影响。将三十天的GCDH（ - / - ）和WT小鼠喂养正常的食物（0.9％Lys），或提交至高Lys饮食（4.7％）72小时。此外，这些动物在不同时间内用三个腹腔注射盐水或L-型汽车施用。 GCDH（ - / - ）小鼠缺乏L-轿车，并提出了更高的谷乳酸碱水平。它们还呈现了脂质和蛋白质损伤，与WT小鼠相比，ROS生产和改变的抗氧化酶。另外，暴露于Lys过载的小鼠在正常饮食下的小鼠呈现出这些参数的更高的改变，这在接受L-Car的那些中显着降低或标准化。因此，我们证明了L-CAR治疗衰减或废除GCH（ - / - ）小鼠氧化应激过程的新有益效果。

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