Proteomic Studies in Inclusion Body Myositis

描述（由申请人提供）：包涵体肌炎（IBM）是一种原因不明的疾病，其特征是缓慢进行性无痛性无力，且无有效治疗。它是一组称为炎性肌病的疾病之一，其中自身免疫的某些方面被认为是突出的。IBM被描述为一种异常蛋白质积累的疾病。 我们建议使用强大的蛋白质分馏和质谱方法对IBM和其他炎症性肌病肌肉中的蛋白质进行详细和大规模的研究。具体的目的是广泛地看在整个范围内的炎症性肌病的肌肉内存在的蛋白质的配置文件;试图从IBM肌肉纯化不溶性蛋白质聚集体，并通过使用质谱表征这些聚集体;和相关的蛋白质组学与微阵列数据。公共卫生相关性。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蛋白质聚集是其机制重要组成部分的疾病的性质。该提案中开发的技术与广泛的生物医学研究相关。

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.