Department of Medical Genetics, Cambridge Institute for Medical Research, University of Cambridge, Cambridge, CB 0XY, UK.
Curr Opin Cell Biol. 2011 Apr;23(2):190-7. doi: 10.1016/j.ceb.2010.10.010. Epub 2010 Nov 17.
A large group of diseases, termed protein misfolding disorders, share the common feature of the accumulation of misfolded proteins. The possibility of a common mechanism underlying either the pathogenesis or therapy for these diseases is appealing. Thus, there is great interest in the role of protein degradation via autophagy in such conditions where the protein is found in the cytoplasm. Here we review the growing evidence supporting a role for autophagic dysregulation as a contributing factor to protein accumulation and cellular toxicity in certain protein misfolding disorders and discuss the available evidence that upregulation of autophagy may be a valuable therapeutic strategy.
一大类疾病被称为蛋白质错误折叠疾病，它们具有蛋白质错误折叠积累的共同特征。在这些疾病的发病机制或治疗中存在共同机制的可能性是吸引人的。因此，人们对蛋白质降解通过自噬在细胞质中发现蛋白质的情况下的作用非常感兴趣。在这里，我们回顾了越来越多的证据支持自噬失调作为导致某些蛋白质错误折叠疾病中蛋白质积累和细胞毒性的因素的作用，并讨论了上调自噬可能是一种有价值的治疗策略的现有证据。
