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首页> 外文期刊>The European Journal of Neuroscience >Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3
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Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3

机译：DNAJB家庭成员（HSP40）的水平与3型脊髓小脑共济失调患者的疾病发作相关

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摘要

In polyglutamine disorders, the length of the expanded CAG repeat shows a strong inverse correlation with the age at disease onset, yet up to 50% of the variation in age of onset is determined by other additional factors. Here, we investigated whether variations in the expression of heat shock proteins (HSP) are related to differences in the age of onset in patients with spinocerebellar ataxia (SCA)3. Hereto, we analysed the protein expression levels of HSPA1A (HSP70), HSPA8 (HSC70), DNAJB (HSP40) and HSPB1 (HSP27) in fibroblasts from patients and healthy controls. HSPB1 levels were significantly upregulated in fibroblasts from patients with SCA3, but without relation to age of onset. Exclusively for expression of DNAJB family members, a correlation was found with the age of onset independent of the length of the CAG repeat expansion. This indicates that DNAJB members might be contributors to the variation in age of onset and underlines the possible use of DNAJB proteins as therapeutic targets.
机译：在聚谷氨酰胺疾病中，CAG重复序列的扩展长度与疾病发作年龄呈强烈反相关，但发病年龄变异中高达50％的变化由其他因素决定。在这里，我们调查了脊髓小脑性共济失调（SCA）3患者热休克蛋白（HSP）表达的变化是否与发病年龄的差异有关。到目前为止，我们分析了来自患者和健康对照组的成纤维细胞中HSPA1A（HSP70），HSPA8（HSC70），DNAJB（HSP40）和HSPB1（HSP27）的蛋白表达水平。 SCA3患者的成纤维细胞中HSPB1水平显着上调，但与发病年龄无关。专门用于表达DNAJB家族成员，发现了与发病年龄的相关性，而与CAG重复扩增的长度无关。这表明DNAJB成员可能是发病年龄变化的原因，并突显了将DNAJB蛋白用作治疗靶点的可能。

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