Molecular pathogenesis of neonatal fulminant dystrophic dog and fetal therapy

营养不良犬是杜氏肌营养不良模型，新生儿期血清肌酸激酶（CK）水平显著升高，死亡率高。通过测量脐带血和静脉血的血清CK水平，我们发现初始呼吸导致大量横膈膜损伤并导致血清CK水平升高。基因芯片显示骨桥蛋白在呼吸作用开始前显著上调，而c-fos、egr-1、IL-6和IL-8在呼吸作用结束后明显过表达。这些分子可能与营养不良肌肉的肌肉损伤有关。

The dystrophic dog, a model of Duchene muscular dystrophy , shows a high mortality rate with a marked increase in serum creatine kinase (CK) levels in the neonatal period. By measuring serum CK levels in cord and venous blood, we found initial respiration resulted in massive diaphragm damage and lead to the high serum CK levels. cDNA microarray revealed that osteopontin was prominently upregulated even prior to the initial respiration and c-fos, egr-1, IL-6, and IL-8 were distinctly overexpressed after the respiration. These molecules could be associated with muscle damage in dystrophic muscle.