Proteomic Studies in Inclusion Body Myositis

描述(申请人提供)：包涵体肌炎(IBM)是一种原因不明的疾病，以缓慢进展的无痛性无力为特征，且没有有效的治疗方法。这是一组被称为炎症性肌病的疾病之一，在这种疾病中，自身免疫的某些方面被认为是突出的。IBM一直被描述为一种异常蛋白质积聚的疾病。 我们建议使用强大的蛋白质分级和质谱学方法对IBM和其他炎症性肌病的肌肉中的蛋白质进行详细和大规模的研究。其具体目标是广泛观察各种炎症性肌病肌肉中存在的蛋白质的图谱；试图从IBM肌肉中提纯不溶性蛋白质聚集体，并通过使用质谱学来表征这些聚集体；以及将蛋白质组学与微阵列数据相关联。与公共卫生相关。蛋白质聚集疾病是重要的公共卫生问题，这项研究有可能进一步了解一种疾病的性质，在这种疾病中，蛋白质聚集是机制的重要组成部分。这项提案中开发的技术与广泛的生物医学研究有关。

DESCRIPTION (provided by applicant): Inclusion body myositis (IBM) is a disease of unknown cause characterized by slowly progressive painless weakness and with no effective treatment. It is one of a group of disorders known as inflammatory myopathies, in which some aspect of autoimmunity is believed to be prominent. IBM has been characterized as a disease in which there is accumulation of abnormal proteins. We propose detailed and large-scale studies of proteins in muscle in IBM and other inflammatory myopathies using powerful protein fractionation and mass spectrometry methods. The specific aims are to look broadly at the profile of proteins present within muscle across the range of inflammatory myopathies; to attempt to purify insoluble protein aggregates from IBM muscle and characterize these aggregates by the use of mass spectrometry; and to correlate proteomic with microarray data. PUBLIC HEALTH RELEVANCE. Diseases of protein aggregation are important public health concerns and this research has the potential to further understand the nature of a disease in which protein aggregation is an important part of the mechanism. The technology developed in this proposal has relevance across a wide range of biomedical research.