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Roles of extracellular chaperones in amyloidosis

机译：细胞外伴侣在淀粉样变性中的作用

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Extracellular protein misfolding and aggregation underlie many of the most serious amyloidoses including Alzheimer's disease, spongiform encephalopathies and type II diabetes. Despite this, protein homeostasis (proteostasis) research has largely focussed on characterising systems that function to monitor protein conformation and concentration within cells. We are now starting to identify elements of corresponding systems, including an expanding family of secreted chaperones, which exist in the extracellular space. Like their intracellular counterparts, extracellular chaperones are likely to play a central role in systems that maintain proteostasis; however, the precise details of how they participate are only just emerging. It is proposed that extracellular chaperones patrol biological fluids for misfolded proteins and facilitate their clearance via endocytic receptors. Importantly, many amyloidoses are associated with dysfunction in rates of protein clearance. This is consistent with a model in which disruption to, or overwhelming of, the systems responsible for extracellular proteostasis results in the accumulation of pathological protein aggregates and disease. Further characterisation of mechanisms that maintain extracellular proteostasis will shed light on why many serious diseases occur and provide us with much needed strategies to combat them.
机译：细胞外蛋白错误折叠和聚集是许多最严重的淀粉样蛋白的基础，其中包括阿尔茨海默氏病，海绵状脑病和II型糖尿病。尽管如此，蛋白质稳态（proteostasis）研究仍主要集中于表征可监测蛋白质构象和细胞内浓度的系统。现在，我们开始确定相应系统的元件，包括存在于细胞外空间中的分泌型伴侣分子的扩大家族。像它们的细胞内对应物一样，细胞外伴侣可能在维持蛋白稳态的系统中起核心作用。但是，关于如何参与的确切细节才刚刚出现。有人提出，细胞外分子伴侣会在生物液体中巡逻，以寻找错误折叠的蛋白质并促进它们通过内吞受体清除。重要的是，许多淀粉样蛋白与蛋白质清除率的功能障碍有关。这与其中导致细胞外蛋白稳态的系统的破坏或压倒导致病理性蛋白质聚集体和疾病的积累的模型是一致的。维持细胞外蛋白稳态的机制的进一步表征将阐明为什么发生许多严重疾病，并为我们提供了与之抗衡的急需策略。

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