INCREASED TURNOVER OF MUSCLE CONTRACTILE PROTEINS IN DUCHENNE MUSCULAR-DYSTROPHY AS ASSESSED BY 3-METHYLHISTIDINE AND CREATININE EXCRETION

被引:53
作者
BALLARD, FJ [1 ]
TOMAS, FM [1 ]
STERN, LM [1 ]
机构
[1] REGENCY PK CTR PHYS HANDICAPPED CHILDREN, REGENCY PK, AUSTRALIA
关键词
D O I
10.1042/cs0560347
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
1. Myofibrillar protein degradation has been measured in patients with Duchenne muscular dystrophy, normal boys, adult males and Duchenne carriers by the rate of 3-methylhistidine excretion after transfer of subjects to a meat-free diet. 2. Although absolute rates of protein breakdown are lower in Duchenne patients, expression of the data to allow for differences in muscle mass gives fractional degradation rates 2-3 times higher than in age-matched controls. 3. Fractional rates of muscle protein synthesis are increased in the Duchenne patients to almost the same extent as protein breakdown. 4. Rates of muscle protein breakdown in obligate and presumed carriers of the Duchenne gene are not different from controls.
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页码:347 / 352
页数:6
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