EXERCISE IN TYPE III GLYCOGEN STORAGE DISEASE

该子项目是利用 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来源获得主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 中心，不一定是研究者的机构。 进行性和经常使人衰弱的肌病是IIIa型糖原累积病（GSDIIIa）患者发病的主要原因。 虽然严重残疾通常不会发生，直到第三至第四个几十年的生活，血清肌酸激酶和醛缩酶浓度升高往往发生在幼儿进行性肌肉损伤一致。运动时肌肉酶的升高和运动的限制可能会减少肌肉损伤的进展。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.