STUDY OF CREATINE METABOLISM IN DISEASES CAUSING MUSCLE WASTING

STUDY OF CREATINE METABOLISM IN DISEASES CAUSING MUSCLE WASTING
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DOI:
10.1172/jci104929
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发表时间:
1964-01-01
影响因子:
15.9
通讯作者:
SINTON, DW
SINTON, DW
中科院分区:
医学1区
文献类型:
--
作者:
FITCH, CD;SINTON, DW

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对于肌肉功能正常的受试者,静脉注射肌酸-1-C14后尿肌酐比活度下降的半衰期为38天;肌萎缩侧索硬化症患者 39 天;迟发性肌病患者 49 天;杜氏肌营养不良症患者的第 13、14 和 21 天;面肩肱型肌营养不良症患者 23 天。在几个时间间隔内,骨骼肌肌酸比活性与尿肌酸酐相似。因此,放射性尿肌酐的消失反映了放射性肌酸从肌肉中的消失。尿肌酸的同位素含量和/或其变化率与杜氏肌营养不良症患者的尿肌酐相同。这些发现表明,杜氏肌营养不良症患者的肌肉无法正常保留肌酸。
The half-time of the decrease in urinary creatinine specific activity after an intravenous injection of creatine-1-C14 was 38 days in a subject with normal muscle function; 39 days in a patient with amyotrophic lateral sclerosis; 49 days in a patient with a myopathy of late onset; 13, 14, and 21 days in patients with Duchenne muscular dystrophy; and 23 days in a patient with facioscapulohumeral muscular dystrophy. Skeletal muscle creatine specific activity was similar to that of urinary creatinine at several time intervals. Thus the disappearance of radioactive urinary creatinine reflects the disappearance of radioactive creatine from muscle. The isotope content of urinary creatine and/or its rate of change was identical to that of urinary creatinine in the patients with Duchenne muscular dystrophy. These findings indicate that muscle is unable to retain creatine normally in Duchenne muscular dystrophy.