肌萎缩的

Objective To explore the clinical value of MRI and MR diffusion tensor imaging in amyotrophic lateral sclerosis.

目的探讨常规MRI检查和MR扩散张量成像新技术在肌萎缩侧索硬化症中的临床应用价值。

Amyotrophic lateral sclerosis is a fatal neurodegenerative disease that is familial in 10% of cases.

肌萎缩性侧束硬化症是一种致命的神经退化性疾病，其家族遗传率是10%。

BACKGROUND: Amyotrophic lateral sclerosis lacks of effective therapeutic method.

背景：肌萎缩侧索硬化尚缺乏有效治疗手段，细胞学治疗是研究的重要方向之一。

A nitrogenous organic acid,C4H9N3O2,that is found in the muscle tissue of vertebrate s mainly in the form of phosphocreatine and supplies energy for muscle contraction.

肌酸一种含氮的有机酸，C4H9N3O2，主要以磷酸肌酸的形式发现于脊椎动物的肌肉细胞组织中，并可为肌肉萎缩提供能量

A nitrogenous organic acid, C4H9N3O2, that is found in the muscle tissue of vertebrates mainly in the form of phosphocreatine and supplies energy for muscle contraction.

肌酸一种含氮的有机酸，c4h9n3o2，主要以磷酸肌酸的形式发现于脊椎动物的肌肉细胞组织中，并可为肌肉萎缩提供能量

The morphologic change of muscle fibers: At the early stage of denervation, the area of cross sections decreased, some muscle fibers turned into angular fibers, nuclei congregated around the center of the fibers. After 8 weeks of denervation, atrophic and hypertrophic fibers coexist. Collagen increases under the endomysium and between the fibers.

肌纤维的组织形态学变化：面部肌肉失神经支配早期，肌纤维的横截面面积变小，出现角状纤维，肌细胞核聚集，细胞核从肌纤维边缘移至肌纤维中央附近；失神经支配后期，肿胀与萎缩的肌纤维并存，肿胀的肌纤维的横截面成倍大于正常的肌纤维，而萎缩的肌纤维的横截面则成倍小于正常的肌纤维；有大量的角纤维存在，肌纤维之间有较多的结缔组织增生。

Background and Objective Pseudohypertrophic muscular dystrophy is a kind of lethal X-chain recessive inherited disease. The etiological factor is mainly due to gene mutation of Xp21 which induce the structural and functional abnormality of a kind of cell skeleton protein: dystrophin.

背景和目的假肥大型肌营养不良症(pseudohypertrophic muscular dystrophy，PMD)是一种常见的致死性X-连锁隐性遗传病，病因主要是由于X染色体短臂2区1带(Xp21)的基因突变而导致一种细胞骨架蛋白——抗肌萎缩蛋白的结构和功能异常所致。

The results show that dystrophin protein was produced in the injected muscle and no serious side-effects were observed.

研究者发现在杜氏进行性肌营养不良患者被注射的肌肉组织中有抗肌萎缩蛋白的表达，同时没有发现不良的副作用。

The treated mice had high levels of dystrophin produced in their muscles, including the heart.

接受治疗的杜氏进行性肌营养不良实验鼠的肌肉细胞中中产生了高水平的抗肌萎缩蛋白的表达。

In most cases of mild Becker muscular dystrophy, the number of deleted nucleotides will be a multiple of three.

绝大多数的贝克型进行性肌营养不良患者抗肌萎缩蛋白基因上缺失的核苷酸是三的倍数。

The split between the two groups can hardly be papered over.

这两个团体间的分歧难以掩饰。

This approach not only encourages a greater number of responses, but minimizes the likelihood of stale groupthink.

这种做法不仅鼓励了更多的反应，而且减少跟风的可能性。