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杜氏肌营养不良症患儿酸溶性肽的尿排泄情况

Urinary excretion of acid-soluble peptides in children with Duchenne muscular dystrophy.

作者信息

Hirano K, Sakamoto Y

机构信息

Faculty of Science of Living, Osaka City University, Japan.

出版信息

Acta Paediatr Jpn. 1994 Dec;36(6):627-31. doi: 10.1111/j.1442-200x.1994.tb03258.x.

DOI:10.1111/j.1442-200x.1994.tb03258.x
PMID:7871971
Abstract

In order to investigate the validity of the hypothesis that acid-soluble peptides (ASP) in urinary excreta can be applied as an index of the protein catabolism of the whole body, we measured the urinary excretion of ASP in 46 normal children and in 18 children with Duchenne muscular dystrophy (DMD), in which continuous breakdown of skeletal muscle protein is presumed. The mean value of ASP in the children with DMD was significantly higher than that in normal controls. The concentration of ASP was correlated with that of 3-methylhistidine (3MH), which has been proposed as an index of muscle breakdown. This finding indicates that urinary ASP reflects the catabolism of body proteins. No correlation was observed between the concentration of ASP and that of 1-methylhistidine (1MH), which is used as an objective index of meat and fish ingestion. After the administration of bestatin, an inhibitor of leucine aminopeptidase, for 9 months, the urinary ASP concentration of children with DMD increased markedly. This increase is thought to have been directly caused by the bestatin itself. Urinary ASP is therefore apparently a more conveniently applied index of protein catabolism than is urinary 3MH, which requires the application of several restrictions. However, it should not be applied when the effect of bestatin administration is evident.

摘要

为了研究尿排泄物中的酸溶性肽(ASP)能否作为全身蛋白质分解代谢指标这一假设的有效性,我们测定了46名正常儿童和18名杜氏肌营养不良症(DMD)患儿的尿ASP排泄情况,推测DMD患儿存在骨骼肌蛋白的持续分解。DMD患儿的ASP平均值显著高于正常对照组。ASP浓度与已被提议作为肌肉分解指标的3 - 甲基组氨酸(3MH)浓度相关。这一发现表明尿ASP反映了机体蛋白质的分解代谢。未观察到ASP浓度与用作肉类和鱼类摄入客观指标的1 - 甲基组氨酸(1MH)浓度之间存在相关性。在给予亮氨酸氨肽酶抑制剂贝司他汀9个月后,DMD患儿的尿ASP浓度显著升高。这种升高被认为是由贝司他汀本身直接引起的。因此,与需要应用一些限制条件的尿3MH相比,尿ASP显然是一种更便于应用的蛋白质分解代谢指标。然而,当贝司他汀给药效果明显时,不应使用尿ASP。

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