MICROIMAGING & SPECTROSCOPY OF MUSCLE DAMAGE

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 在营养不良性肌病的整个进展过程中，受影响的肌肉组织经历了 由于它们对损伤的高度敏感性，退化和再生的许多周期。 当这些肌肉的再生能力耗尽时，受损的纤维会被破坏。 逐渐被脂肪和结缔组织取代。在这种高度脆弱性的基础上， 损伤是肌营养不良蛋白或肌营养不良蛋白相关糖蛋白（DAG）的缺陷。以来 病理作用通常是由于缺乏单一蛋白质，肌营养不良症是 非常适合于基因治疗、干细胞治疗以及药物治疗。电流 监测治疗干预和诊断的功效的方法严重依赖于组织 活检和组织学。非侵入性成像模式的发展是非常必要的 评价新的治疗方法恢复肌营养不良蛋白/DAG的能力， 复杂.我们已经发现，在磁化传递特性的显着差异， 健康和纤维化的营养不良肌肉，其可以在基因转移后逆转。 这些 结果表明，磁化传递对比度可以提供一种非侵入性的测量， 疾病进展和营养不良的治疗干预。

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.