Iannaccone S T, Nagy B, Samaha F J
J Child Neurol. 1987 Jan;2(1):17-21. doi: 10.1177/088307388700200103.
Muscle cells were cultured from six patients with Duchenne muscular dystrophy and nine normal subjects. Protein and myosin content and pyruvate kinase (PK) activity were similar in normal and Duchenne muscular dystrophy cultures. Creatine kinase (CK) activity was lower in Duchenne muscular dystrophy cultures and the isoenzyme distribution indicated MB-CK was significantly lower, while BB-CK was significantly higher in later Duchenne muscular dystrophy cultures. This abnormal isoenzyme pattern suggested aberrant or impaired maturation of Duchenne muscular dystrophy myotubes in vitro.
从6名杜氏肌营养不良患者和9名正常受试者中培养出肌肉细胞。正常培养物和杜氏肌营养不良培养物中的蛋白质、肌球蛋白含量以及丙酮酸激酶(PK)活性相似。杜氏肌营养不良培养物中的肌酸激酶(CK)活性较低,同工酶分布表明,在杜氏肌营养不良后期培养物中,MB-CK显著降低,而BB-CK显著升高。这种异常的同工酶模式表明杜氏肌营养不良肌管在体外成熟异常或受损。