Department of Biochemistry and Biophysics, University of California San Francisco, San Francisco, California, United States of America.
PLoS Biol. 2010 Aug 10;8(8):e1000450. doi: 10.1371/journal.pbio.1000450.
Aberrant protein aggregation is a hallmark of many age-related diseases, yet little is known about whether proteins aggregate with age in a non-disease setting. Using a systematic proteomics approach, we identified several hundred proteins that become more insoluble with age in the multicellular organism Caenorhabditis elegans. These proteins are predicted to be significantly enriched in beta-sheets, which promote disease protein aggregation. Strikingly, these insoluble proteins are highly over-represented in aggregates found in human neurodegeneration. We examined several of these proteins in vivo and confirmed their propensity to aggregate with age. Different proteins aggregated in different tissues and cellular compartments. Protein insolubility and aggregation were significantly delayed or even halted by reduced insulin/IGF-1-signaling, which also slows aging. We found a significant overlap between proteins that become insoluble and proteins that influence lifespan and/or polyglutamine-repeat aggregation. Moreover, overexpressing one aggregating protein enhanced polyglutamine-repeat pathology. Together our findings indicate that widespread protein insolubility and aggregation is an inherent part of aging and that it may influence both lifespan and neurodegenerative disease.
异常蛋白聚集是许多与年龄相关疾病的标志,但对于在非疾病状态下蛋白质是否会随年龄增长而聚集知之甚少。本研究采用系统蛋白质组学方法,在多细胞生物秀丽隐杆线虫中鉴定出数百种随年龄增长而变得更不易溶解的蛋白质。这些蛋白质被预测在β-折叠中显著富集,从而促进疾病蛋白聚集。引人注目的是,这些不溶性蛋白质在人类神经退行性疾病中发现的聚集体中高度过表达。我们在体内研究了其中几种蛋白质,并证实它们随着年龄的增长而易于聚集。不同的蛋白质在不同的组织和细胞区室中聚集。胰岛素/IGF-1 信号的降低显著延迟或甚至阻止了蛋白质的不溶性和聚集,而胰岛素/IGF-1 信号的降低也会减缓衰老。我们发现,变得不溶性的蛋白质与影响寿命和/或多聚谷氨酰胺重复聚集的蛋白质之间存在显著重叠。此外,过表达一种聚集蛋白会增强多聚谷氨酰胺重复病理。总之,我们的研究结果表明,广泛的蛋白质不溶性和聚集是衰老的固有组成部分,它可能影响寿命和神经退行性疾病。