Greenberg Steven A
Children's Hospital Informatics Program, Department of Neurology, Division of Neuromuscular Disease, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA.
Curr Opin Neurol. 2009 Oct;22(5):516-23. doi: 10.1097/WCO.0b013e3283311ddf.
Recent developments pertaining to disease mechanisms in the inflammatory myopathies are discussed, emphasizing those areas that are of particular interest to me.
The identification and further characterization of the type 1 interferon pathway in dermatomyositis is leading down a path of genomic medicine. Myonuclear structural abnormalities and the presence of nucleic acid-binding proteins, including the TAR DNA binding protein TDP-43, in sporadic inclusion body myositis (sIBM) sarcoplasm are important recent observations. This is an area likely to provide deep understanding of the mechanism of myofiber injury in sIBM. Proteomic characterization of proteins in sIBM muscle, muscle functioning as a lymphoid tissue, and the nature of belief systems, particularly one pertaining to beta-amyloid and sIBM, are other areas of interest.
Clarification of disease mechanisms is providing a basis for rational drug development for some patients with myositis.
讨论了炎症性肌病疾病机制的最新进展，重点强调了我特别感兴趣的领域。
皮肌炎中1型干扰素途径的鉴定及进一步特征分析正引领着基因组医学的发展道路。散发性包涵体肌炎（sIBM）肌浆中肌核结构异常以及核酸结合蛋白（包括TAR DNA结合蛋白TDP-43）的存在是近期的重要观察结果。这一领域可能会深入揭示sIBM中肌纤维损伤的机制。sIBM肌肉中蛋白质的蛋白质组学特征、肌肉作为淋巴组织的功能以及信念系统的本质，特别是与β-淀粉样蛋白和sIBM相关的信念系统，是其他感兴趣的领域。
疾病机制的阐明为一些肌炎患者的合理药物开发提供了依据。
