Glesby M J, Rosenmann E, Nylen E G, Wrogemann K
Department of Biochemistry, University of Manitoba, Winnipeg, Canada.
Muscle Nerve. 1988 Aug;11(8):852-6. doi: 10.1002/mus.880110809.
Serum creatine kinase (CK) activity, calcium (Ca) and magnesium (Mg) contents of skeletal muscle and isolated mitochondria, as well as oxidative phosphorylation of X-linked muscular dystrophic (mdx) mice were compared with normal control animals at ages 5, 10, and 23 weeks. Serum CK is elevated in mdx mice at all ages, with highest activities at 5 weeks. The Ca content of dystrophic skeletal muscle is increased at all ages, whereas no clearly abnormal trend in muscle Mg levels was observed. Noncollagen protein (NCP), which was used as a reference base, is significantly diminished in muscle from 10- and 23-week-old mdx animals. Isolated mitochondria from mdx mice have elevated calcium content and decreased respiratory control ratios with NAD-linked substrates pyruvate/malate. The findings are distinct from those in dystrophic mice, strain 129/ReJ, but similar to observations in dystrophic hamsters and Duchenne muscular dystrophy and reflect the occurrence of overt muscle cell necrosis.
在5周、10周和23周龄时,将X连锁型肌营养不良(mdx)小鼠的血清肌酸激酶(CK)活性、骨骼肌和分离线粒体中的钙(Ca)和镁(Mg)含量,以及氧化磷酸化情况与正常对照动物进行了比较。mdx小鼠在所有年龄段的血清CK均升高,在5周龄时活性最高。营养不良的骨骼肌中钙含量在所有年龄段均增加,而未观察到肌肉镁水平有明显异常趋势。用作参考基准的非胶原蛋白(NCP)在10周龄和23周龄mdx动物的肌肉中显著减少。来自mdx小鼠的分离线粒体钙含量升高,与NAD连接底物丙酮酸/苹果酸的呼吸控制率降低。这些发现与129/ReJ品系的营养不良小鼠不同,但与营养不良仓鼠和杜氏肌营养不良的观察结果相似,反映了明显的肌肉细胞坏死的发生。