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首页> 外文期刊>Neurobiology of disease >Progressive dysfunction of the cholesterol biosynthesis pathway in the R6/2 mouse model of Huntington's disease.
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Progressive dysfunction of the cholesterol biosynthesis pathway in the R6/2 mouse model of Huntington's disease.

机译：亨廷顿病的R6 / 2小鼠模型中胆固醇生物合成途径的进行性功能障碍。

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We have recently reported significantly reduced levels of the mRNA of genes critical for the cholesterol biosynthesis pathway in the brains of mice and patients with Huntington's disease (HD), which are indicative of a biological dysfunction. We here show that the brains of R6/2 transgenic mice have progressively decreasing levels of the cholesterol precursors, lathosterol and lanosterol, and declining 3-hydroxy-3-methylglutaryl coenzyme A reductase activity starting from pre-symptomatic stages. We also show that, despite the progressive reduction of brain cholesterol biosynthesis, steady-state levels of total cholesterol remain constant, thus suggesting that compensatory mechanisms are in operation. These in vivo findings indicate a consistent and progressive reduction in the activity of the cholesterol biosynthesis pathway in HD brain. The defect occurs early in these mice and generates lower levels of newly synthesized cholesterol and its intermediates, which may affect different aspects of the disease.
机译：最近，我们报道了小鼠和患有亨廷顿舞蹈病（HD）的患者大脑中对于胆固醇生物合成途径至关重要的基因的mRNA水平显着降低，这表明生物学功能异常。我们在这里显示R6 / 2转基因小鼠的大脑具有逐渐降低的胆固醇前体，谷甾醇和羊毛甾醇的水平，并从有症状的阶段开始降低3-羟基-3-甲基戊二酰辅酶A的还原酶活性。我们还显示，尽管脑胆固醇生物合成逐渐减少，但总胆固醇的稳态水平保持恒定，因此表明补偿机制正在发挥作用。这些体内发现表明，HD脑中胆固醇生物合成途径的活性持续不断地降低。缺陷在这些小鼠中较早发生，并产生较低水平的新合成胆固醇及其中间体，这可能会影响疾病的不同方面。

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