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包涵体肌炎:从秀丽隐杆线虫肌肉角度的观察

Inclusion body myositis: a view from the Caenorhabditis elegans muscle.

作者信息

Rebolledo Daniela L, Minniti Alicia N, Grez Paula M, Fadic Ricardo, Kohn Rebecca, Inestrosa Nibaldo C

机构信息

Centro de Envejecimiento y Regeneración (CARE), Centro de Regulación Celular y Patología Joaquín V. Luco, MIFAB, Facultad de Ciencias Biológicas, P. Universidad Católica de Chile, Alameda 340, Santiago, Chile.

出版信息

Mol Neurobiol. 2008 Oct;38(2):178-98. doi: 10.1007/s12035-008-8041-0. Epub 2008 Sep 5.

Abstract

Inclusion body myositis (IBM) is the most common myopathy in people over 50 years of age. It involves an inflammatory process that, paradoxically, does not respond to anti-inflammatory drugs. A key feature of IBM is the presence of amyloid-beta-peptide aggregates called amyloid deposits, which are also characteristic of Alzheimer's disease. The use of animals that mimic at least some characteristics of a disease has become very important in the quest to elucidate the molecular mechanisms underlying this and other pathogeneses. Although there are some transgenic mouse strains that recreate some aspects of IBM, in this review, we hypothesize that the great degree of similarity between nematode and human genes known to be involved in IBM as well as the considerable conservation of biological mechanisms across species is an important feature that must be taken into consideration when deciding on the use of this nematode as a model. Straightforward laboratory techniques (culture, transformation, gene knockdown, genetic screenings, etc.) as well as anatomical, physiological, and behavioral characteristics add to the value of this model. In the present work, we review evidence that supports the use of Caenorhabditis elegans as a biological model for IBM.

摘要

包涵体肌炎(IBM)是50岁以上人群中最常见的肌病。它涉及一种炎症过程,矛盾的是,这种炎症过程对抗炎药物没有反应。IBM的一个关键特征是存在称为淀粉样沉积物的β-淀粉样肽聚集体,这也是阿尔茨海默病的特征。在试图阐明这种疾病和其他发病机制的分子机制时,使用至少模拟疾病某些特征的动物变得非常重要。尽管有一些转基因小鼠品系重现了IBM的某些方面,但在本综述中,我们假设线虫与已知参与IBM的人类基因之间的高度相似性以及物种间生物机制的相当大的保守性是在决定使用这种线虫作为模型时必须考虑的一个重要特征。简单的实验室技术(培养、转化、基因敲低、基因筛选等)以及解剖学、生理学和行为特征增加了这个模型的价值。在本工作中,我们综述了支持秀丽隐杆线虫作为IBM生物学模型的证据。

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