Inclusion-body myositis: newest concepts of pathogenesis and relation to aging and Alzheimer disease.
Inclusion-body myositis: newest concepts of pathogenesis and relation to aging and Alzheimer disease.
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包涵体肌炎：发病机制的最新概念以及与衰老和阿尔茨海默病的关系。
DOI：
10.1093/jnen/60.1.1
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发表时间：
2001
影响因子：
3.2
通讯作者：
Engel,WK
中科院分区：
文献类型：
--
作者：
Askanas,V;Engel,WK
我们回顾了有关散发性包涵体肌炎发病机制(S)研究的最新进展，并提出了S-IBM的病理诊断标准。我们讨论了以下几个主题的可能致病作用：1)淀粉样蛋白前体蛋白(A-βPP)及其片段A-βPP的增加；2)tau蛋白的磷酸化；3)氧化应激；4)异常的a)信号转导、b)转录和c)核糖核酸积累；5)“连接”和“肌源性”失神经；以及6)淋巴细胞性炎症。有证据支持我们的假设，即AβPP在衰老的肌肉纤维中的过度表达是导致随后的致病级联的早期上游事件。讨论了S-IBM肌与阿尔茨海默病(AD)脑在病理上的显著相似性，并讨论了其可能的原因和意义。
We review the newest advances related to seeking the pathogenic mechanism(s) of sporadic inclusion-body myositis (s-IBM) and present the pathologic diagnostic criteria of s-IBM. We discuss the possible pathogenic role of several themes, such as 1) increased amyloid-β precursor protein (AβPP) and of its fragment Aβ; 2) phosphorylation of tau protein; 3) oxidative stress; 4) abnormal a) signal-transduction, b) transcription, and c) RNA accumulation; 5) “junctionalization” and “myogenous” denervation; and 6) lymphocytic inflammation. Evidence is provided supporting our hypothesis that overexpression of AβPP within the aging muscle fibers is an early upstream event causing the subsequent pathogenic cascade. The remarkable pathologic similarities between s-IBM muscle and Alzheimer disease (AD) brain are discussed, and the possible cause and significance are addressed.