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[U-14C]葡萄糖掺入杜兴氏肌营养不良症患者和对照患者肌肉中的中性脂质和sn-甘油-3-磷酸的情况。

Incorporation of [U-14C]glucose into neutral lipids and sn-glycerol-3-phosphate in muscle from Duchenne muscular dystrophy and control patients.

作者信息

West D P, Ellis D A, Strickland J M

出版信息

J Neurol Sci. 1977 Aug;33(1-2):131-42. doi: 10.1016/0022-510x(77)90187-3.

DOI:10.1016/0022-510x(77)90187-3
PMID:903778
Abstract

Muscle biopsy tissue from patients suffering from a wide range of neuromuscular disorders, normal muscle removed during regular orthopaedic procedures, and muscle from aborted human fetuses of 12--20 weeks gestation was dissected into fibre-bundles and incubated at 37 degrees C with [U-14C]glucose at a final concentration of 4 mM. Extracts made: (i) after 30 min were used to determine content and radioactivity of sn-glycerol-3-phosphate, and (ii) after 160 min for separation of lipid species. Dystrophic muscle from 8 patients with typical Duchenne dystrophy converted significantly more glucose than normal muscle (11 samples) into neutral lipids, but less into polar lipids. Of total lipid radioactivity a mean value of 66% was found in neutral lipid in the Duchenne group, while in various control groups this proportion was about 40%. The disparity observed was the result of greater incorporation of glucose label into triglycerides, chiefly in the glycerol moiety of the Duchenne group lipids, and was some 3 times greater than normal. Other muscle disease groups also showed slightly raised levels of incorporation but these were not statistically significant in the present series of results. The content and radioactivity of glycerol phosphate in muscle from 15 patients with Duchenne dystrophy were also significantly higher than in normal muscle. These findings are discussed in relation to glucose metabolism in muscle and the energy cost of transforming glucose into triglyceride.

摘要

从患有多种神经肌肉疾病的患者身上获取的肌肉活检组织、在常规骨科手术中切除的正常肌肉以及妊娠12 - 20周的人工流产胎儿的肌肉,被切成纤维束,并在37℃下与终浓度为4 mM的[U - 14C]葡萄糖一起孵育。提取物制作如下:(i) 30分钟后用于测定sn - 甘油-3 - 磷酸的含量和放射性,(ii) 160分钟后用于分离脂质种类。8例典型杜兴氏肌营养不良症患者的营养不良肌肉将显著多于正常肌肉(11个样本)的葡萄糖转化为中性脂质,但转化为极性脂质的较少。在杜兴氏组中,中性脂质中脂质总放射性的平均值为66%,而在各个对照组中这一比例约为40%。观察到的差异是由于葡萄糖标记物更多地掺入甘油三酯中,主要是在杜兴氏组脂质的甘油部分,且比正常情况大约高3倍。其他肌肉疾病组也显示掺入水平略有升高,但在本系列结果中这些差异无统计学意义。15例杜兴氏肌营养不良症患者肌肉中的磷酸甘油含量和放射性也显著高于正常肌肉。本文结合肌肉中的葡萄糖代谢以及将葡萄糖转化为甘油三酯的能量消耗对这些发现进行了讨论。

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Incorporation of [U-14C]glucose into neutral lipids and sn-glycerol-3-phosphate in muscle from Duchenne muscular dystrophy and control patients.[U-14C]葡萄糖掺入杜兴氏肌营养不良症患者和对照患者肌肉中的中性脂质和sn-甘油-3-磷酸的情况。
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引用本文的文献

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Decreased lysosomal dipeptidyl aminopeptidase I activity in cultured human skin fibroblasts in Duchenne's muscular dystrophy.杜兴氏肌营养不良症患者培养的人皮肤成纤维细胞中溶酶体二肽基氨基肽酶I活性降低。
J Clin Invest. 1980 Jun;65(6):1398-406. doi: 10.1172/JCI109804.