Carrell R W, Gooptu B
Department of Haematology Cambridge Institute for Medical Research University of Cambridge Hills Road Cambridge CB2 2XY UK. rwc
Curr Opin Struct Biol. 1998 Dec;8(6):799-809. doi: 10.1016/s0959-440x(98)80101-2.
Some of the most perplexing disorders in medicine are each now known to arise from the conformational instability of an underlying protein. The consequence is a continuum of pathologies with typically a change in fold leading to ordered aggregation and tissue deposition. The serpins provide a structural prototype for these pathologies and give a perspective on the assessment of current proposals as to the conformational basis of both Alzheimer's disease and the transmissible prion encephalopathies.
医学上一些最令人困惑的病症现在已知是由一种潜在蛋白质的构象不稳定性引起的。其结果是一系列病理状况,通常伴随着折叠变化,导致有序聚集和组织沉积。丝氨酸蛋白酶抑制剂为这些病理状况提供了一个结构原型,并为评估目前关于阿尔茨海默病和传染性朊病毒脑病构象基础的提议提供了一个视角。