Protein homeostasis in models of aging and age-related conformational disease.
Protein homeostasis in models of aging and age-related conformational disease.
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DOI：
10.1007/978-1-4419-7002-2_11
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发表时间：
2010
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中科院分区：
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蛋白质组的稳定性对于细胞的健康至关重要，并且对生物体的寿命有重大贡献。衰老和许多与年龄相关的疾病都有错误折叠和受损蛋白质表达的共同点。疾病期间受损蛋白质的长期表达会对蛋白质稳态（蛋白质稳态）产生毁灭性后果，导致许多生物过程的破坏。本章讨论了我们目前对蛋白质错误折叠的各种影响因素的理解，以及压力和衰老加速错误折叠以及伴随的聚集/毒性的机制。无脊椎动物模型有助于研究与疾病相关蛋白质的聚集和毒性相关的过程，以及蛋白质稳态失调如何导致衰老神经退行性疾病。
The stability of the proteome is crucial to the health of the cell, and contributes significantly to the lifespan of the organism. Aging and many age-related diseases have in common the expression of misfolded and damaged proteins. The chronic expression of damaged proteins during disease can have devastating consequences on protein homeostasis (proteostasis), resulting in disruption of numerous biological processes. This chapter discusses our current understanding of the various contributors to protein misfolding, and the mechanisms by which misfolding, and accompanied aggregation/toxicity, is accelerated by stress and aging. Invertebrate models have been instrumental in studying the processes related to aggregation and toxicity of disease-associated proteins and how dysregulation of proteostasis leads to neurodegenerative diseases of aging.