The deposition of proteins of abnormal conformation is one of the major hallmarks of the common neurodegenerative diseases including Alzheimer's, Parkinson's, amyotrophic lateral sclerosis, frontotemporal dementia, and prion diseases. Protein quality control systems have evolved to protect cells and organisms against the harmful consequences of abnormally folded proteins that are constantly produced in small amounts. Mutations in rare inherited forms of neurodegenerative diseases have provided compelling evidence that failure of protein quality control systems can drive neurodegeneration. With extensive knowledge of these systems, and the notion that protein quality control may decline with age, many laboratories are now focussing on manipulating these evolutionarily optimized defence mechanisms to reduce the protein misfolding burden for therapeutic benefit.

中文翻译：

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操纵蛋白质质量控​​制系统在神经退行性疾病中的潜在益处。

异常构象蛋白质的沉积是常见神经退行性疾病的主要标志之一，包括阿尔茨海默病、帕金森病、肌萎缩侧索硬化症、额颞叶痴呆和朊病毒病。蛋白质质量控​​制系统已经发展到可以保护细胞和生物体免受不断少量产生的异常折叠蛋白质的有害后果。神经退行性疾病的罕见遗传形式的突变提供了令人信服的证据，表明蛋白质质量控​​制系统的失败可能会导致神经退行性变。凭借对这些系统的广泛了解，以及蛋白质质量控​​制可能随着年龄的增长而下降的概念，许多实验室现在专注于操纵这些进化优化的防御机制，以减少蛋白质错误折叠负担以获得治疗益处。