MICROIMAGING & SPECTROSCOPY OF MUSCLE DAMAGE

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 在营养不良肌病的整个发展过程中，受影响的肌肉组织经历 由于它们对损伤的高度敏感性，导致了多次退化和再生周期。 当这些肌肉的再生能力耗尽时，受损的纤维 逐渐被脂肪和结缔组织取代。这一提升的漏洞是 损害是肌营养不良蛋白或肌营养不良蛋白相关糖蛋白(DAGs)的缺陷。自.以来 病理效应通常是由于缺乏单一蛋白质，肌肉营养不良是 非常适合于基因治疗、干细胞治疗以及药物治疗。当前 监测治疗干预和诊断疗效的方法严重依赖于组织 活检和组织学检查。发展非侵入性成像方式是非常必要的。 评估新的治疗方法恢复dystrophin/DAG的能力 很复杂。我们发现在磁化传递特性上存在显著差异 健康和纤维性营养不良的肌肉，基因转移后可以逆转。这些 结果表明，磁化传递对比可提供一种无创的测量方法。 营养不良的疾病进展和治疗干预。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Throughout the progression of dystrophic myopathies, the affected muscle tissues undergo numerous cycles of degeneration and regeneration due to their high susceptibility to injury. Upon exhaustion of the regenerative capacity of these muscles, damaged fibers are progressively replaced by fat and connective tissue. Underlying this elevated vulnerability to damage is a deficiency in dystrophin or dystrophin associated glycoproteins (DAGs). Since the pathological effects are often due to the lack of a single protein, the muscular dystrophies are well suited for gene therapy, stem cell therapy, as well as pharmaceutical treatment. Current methods of monitoring efficacy of therapeutic intervention and diagnosis rely heavily on tissue biopsy and histology. The development of non-invasive imaging modalities is greatly needed to evaluate novel therapeutic approaches in their ability to restore the dystrophin/DAG complex. We have found significant differences in magnetization transfer characteristics of healthy and fibrotic dystrophic muscles which can be reversed following gene transfer. These results indicate that magnetization transfer contrast may provide a noninvasive measure of disease progression and therapeutic intervention for the dystrophies.