Misfolding of membrane proteins in health and disease: the lady or the tiger?

Misfolding of membrane proteins in health and disease: the lady or the tiger?
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DOI:
10.1016/s0959-440x(00)00112-3
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发表时间:
2000-08-01
影响因子:
6.8
通讯作者:
Nagy, JK
Nagy, JK
中科院分区:
生物学2区
文献类型:
--
作者:
Sanders, CR;Nagy, JK

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蛋白质错误折叠越来越多地被认为是许多疾病的一个因素,包括囊性纤维化,帕金森氏症,阿尔茨海默氏症和动脉粥样硬化。许多涉及错误折叠的病理学的蛋白质是膜相关的,因此双层可以在正常和异常折叠中发挥作用。可以认为,真核生物膜蛋白在体内的折叠和错误折叠途径之间的分配是在动力学控制下的。此外,这些途径之间的平衡可能是惊人的微妙。
Protein misfolding is increasingly recognized as a factor in many diseases, including cystic fibrosis, Parkinson's, Alzheimer's and atherosclerosis. Many proteins involved in misfolding-based pathologies are membrane-associated, such that the bilayer may play roles in normal and aberrant folding. It can be argued that the in vivo partitioning of eukaryotic membrane proteins between folding and misfolding pathways is under kinetic control. Moreover, the balance between these pathways can be surprisingly delicate.