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蛋白质空间构像改变,导致其功能及结构异常,并堆积形成包涵体,引起细胞结构与功能异常。包涵体肌病(IBM)的特征性病理改变——肌浆内或核内包涵体,是多种异常蛋白质堆积的结果。其中,AβPP/Aβ蛋白表达过多和堆积是导致IBM发病的关键因素;Aβ及AβPP堆积使蛋白酶体和多种蛋白质结构与功能异常,进而促进包涵体形成;帕金森病相关蛋白α-synuclein及parkin可能参与IBM发病机制;Myostatin表达增加使IBM的肌肉萎缩程度进一步加重。
Conformational changes in the protein space, resulting in its functional and structural abnormalities, and the accumulation of the formation of inclusion bodies, causing cell structure and function abnormalities. The characteristic pathological changes of inclusion body myopathy (IBM), the intrasternal or intranuclear inclusions, are the result of the accumulation of many abnormal proteins. Among them, AβPP / Aβprotein over-expression and accumulation are the key factors leading to the onset of IBM; Aβ and AβPP accumulation make the proteasome and a variety of protein structure and function abnormalities, thereby promoting the formation of inclusion bodies; Parkinson’s disease associated proteinα-synuclein and Parkin may be involved in the pathogenesis of IBM; increased Myostatin expression further aggravates IBM’s muscle atrophy.