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首页> 外文期刊>Neuron >The unfolded protein response modulates disease severity in pelizaeus-merzbacher disease.
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The unfolded protein response modulates disease severity in pelizaeus-merzbacher disease.

机译：展开的蛋白质反应调节了pelizaeus-merzbacher病的疾病严重性。

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摘要

The unfolded protein response (UPR) is a eukaryotic signaling pathway linking protein flux through the endoplasmic reticulum to transcription and translational repression. Herein, we demonstrate UPR activation in the leukodystrophy Pelizaeus-Merzbacher disease (PMD) as well as in three mouse models of this disease and transfected fibroblasts expressing mutant protein. The CHOP protein, widely known as a proapoptotic transcription factor, modulates pathogenesis in the mouse models of PMD; however, this protein exhibits antiapoptotic activity. Together, these data show that the UPR has the potential to modulate disease severity in many cells expressing mutant secretory pathway proteins. Thus, PMD represents the first member of a novel class of disparate degenerative diseases for which UPR activation and signaling is the common pathogenic mechanism.
机译：展开的蛋白质反应（UPR）是一种真核信号通路，将通过内质网的蛋白质通量与转录和翻译抑制联系起来。在这里，我们证明白细胞营养不良性白细胞-梅尔茨巴赫病（PMD）以及该病的三种小鼠模型和表达突变蛋白的转染成纤维细胞均具有UPR激活作用。 CHOP蛋白被广泛称为促凋亡转录因子，可调节PMD小鼠模型的发病机制。但是，这种蛋白质具有抗凋亡活性。总之，这些数据表明，UPR具有调节表达突变型分泌途径蛋白的许多细胞中疾病严重性的潜力。因此，PMD代表了新型异类退行性疾病的第一个成员，其UPR激活和信号传导是其常见的致病机制。

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