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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Targeting expression of expanded polyglutamine proteins to the endoplasmic reticulum or mitochondria prevents their aggregation.
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Targeting expression of expanded polyglutamine proteins to the endoplasmic reticulum or mitochondria prevents their aggregation.

机译：将扩增的聚谷氨酰胺蛋白表达靶向内质网或线粒体可防止其聚集。

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摘要

Aggregation of misfolded proteins is a characteristic of several neurodegenerative diseases. The huntingtin amino-terminal fragment with extended polyglutamine repeat forms aggregates closely associated with chaperones both in the cytoplasm and the nucleus. Because each cellular compartment contains distinct chaperones and because the molecular mechanisms controlling polyglutamine aggregation are largely unknown, we decided to investigate the influence of different cellular environments on the aggregation of this pathological protein. Here, we show that aggregation of a protein containing a polyglutamine stretch of pathological length is abolished when its expression is targeted to the endoplasmic reticulum. Once retrogradely transported outside the endoplasmic reticulum, the aggregation-prone polyglutamine-containing protein recovers its ability to aggregate. When expressed in the mitochondria, a protein containing 73 glutamines is entirely soluble, whereas the nucleocytosolic equivalent has an extremely high tendency to aggregate. Our data imply that polyglutamine aggregation is a property restricted to the nucleocytosolic compartment and suggest the existence of compartment-specific cofactors promoting or preventing aggregation of pathological proteins.
机译：错误折叠的蛋白质的聚集是几种神经退行性疾病的特征。具有延伸的聚谷氨酰胺重复序列的亨廷顿蛋白氨基末端片段在细胞质和细胞核中均形成与伴侣密切相关的聚集体。由于每个细胞区室都包含不同的分子伴侣，并且由于控制聚谷氨酰胺聚集的分子机制尚不清楚，因此我们决定研究不同细胞环境对该病理蛋白聚集的影响。在这里，我们表明，当表达针对内质网时，含有病理长度的聚谷氨酰胺片段的蛋白质的聚集将被消除。一旦逆行转运到内质网外，含聚谷氨酰胺的易于聚集的蛋白质将恢复其聚集能力。当在线粒体中表达时，含有73个谷氨酰胺的蛋白质完全可溶，而核苷等效物具有极高的聚集趋势。我们的数据表明，聚谷氨酰胺聚集是一种仅限于胞质区室的特性，并暗示了区室特异性辅因子的存在，可促进或阻止病理性蛋白的聚集。

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