EXERCISE IN TYPE III GLYCOGEN STORAGE DISEASE

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。 进行性和经常衰弱的肌病是IIIa型糖原储存病(GSDIIIa型)患者发病的主要原因。虽然严重残疾通常直到生命的第3至第4年才会发生，但血清肌酸激酶和醛缩酶浓度的升高通常发生在进行性肌肉损伤的幼儿中。运动过程中肌酶升高加剧，限制运动可能会减缓肌肉损伤的进展。这项研究的目的是更好地了解运动相关肌肉损伤的原因，并测试预防它的方法。

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. Progressive and frequently debilitating myopathy is the major cause of morbidity in patients with type IIIa glycogen storage disease (GSDIIIa). While severe disability usually does not occur until the 3rd to 4th decades of life, elevations in serum creatine kinase and aldolase concentrations often occur in young children consistent with progressive muscle damage. The elevation in muscle enzymes worsens during exercise, and restriction of exercise may decrease the progression of muscle damage. The goal of this study is to better understand the cause of exercise-related muscle injury and to test a method to prevent it.