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Gene mutations were detected in 25 patients and a 18-year-old girl among 16 families. Trinucleotide repeats of CAG were 73~79. The fragments of abnormal alleles were 380~402bp, and all patients were heterozygous. The copy numbers of normal alleles were 18~40, fragments from 202~270bp. SCV reduction was much obvious compared to MCV, MCV and SCV in lower limb ?ere much more slow than that in upper's. BAEP, VEP were also delayed in latency. The anticipation in parental sex bias were much more obvioius than that in matental's. Cerebellar ataxia was most severe, the next were dysarthria and bulging eyes. Amytrophy was seen only in bed ridden patients. Cerebellar atrophy was more severe than brain stem, cord atrophy was n't observed in all MJD.

结果检出10个家系25例病人及1例症状前18岁女孩有MJD基因突变,CAG三核苷酸重复73~79次,异常等位基因片段长380~402bp,均为杂合子;正常人CAG三核苷酸重复18~40次,等位片段长200~270bp,电生理发现MJD的SCV减慢比MCV明显,而下肢的MCV、SCV又较上肢明显,BAEP、VEP均有不同程度的潜伏期延长或波的异常;MJD的父亲遗传早于母亲,进展也较块,临床以小脑性共济失调为突出症状,其次为构音障碍、突眼等,肌肉萎缩仅见于晚期病人;MRI示小脑萎缩较明显,脑干萎缩并不严重,未见明显的颈髓萎缩。

According to a lasting poll,more and more people show a stong desire for earing more money though other jobs or overtime.

根据最近的一项调查,越来越多的人表达了想从事另外的工作或加班以赚取更多的钱来补贴家用的强烈愿望。

Besides meeting the common requisition for furniture, Furniture in SOHO should be more integrate, more flexible and more distinctive. So that SOHO worker can work easefully and tender-heartedly in home.

SOHO家具既要符合家具的统一要求,如符合人体工程学,同时更具有一体化、高度整合多样功能、更大的灵活性、突出的个性,适应家庭生活的舒适性、随意性,营造居家办公温馨、休闲的氛围。

As he began to earn more and more money, his personality becamemore and more egotistic.

他的钱越赚越多时,他的个性也变得越来越自负。

The more you sleep, the more HGH your body will produce, which results in more elastin in your skin and less vulnerability to wrinkles.

你睡得越多,你的身体会产生越多的生长激素,使你的皮肤而富有更多的弹性,减少出现皱纹的脆弱性。

More legroom, more elbowroom, more comfortable.

更大的放脚空间

More legroom , more elbowroom , more comfortable .

更大的空间放脚,更大的空间扶手,更舒适的环境

Each level awards you attribute points to spend improving your attributes, like the Warrior's Swordsmanship, which increases sword damage; the Monk's Healing Prayers, which makes healing skills more powerful; and the Elementalist's Energy Storage, which permanently increases maximum Energy, allowing you to use more skills more often.

每个职业都有自身的特技,您可以逐步建立最适合您的技能体系每一级您都会获得特技点来强化您的特技,比如战士的剑术会提高剑的伤害;僧侣的治疗祷言会提高治疗效果;元素使的能量储备会提升能量上限,让您尽可能多地使用法术。

What the self-educated man learns, becomes more thoroughly his own, makes a more vivid impression upon his mind, and fixes itself more enduringly there.

一个自学成才的人所获取的知识可以更充分地内化为自己的学识,可以在心中留下更加生动的印象,而且记忆起来更加持久。

More and more severe environmental pollution influences our life, and we should pay more attention to environmental protection.

越来越严重的环境污染影响了我们的生活,我们理当应该更加重视环境保护问题。

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推荐网络例句

Finally, according to market conditions and market products this article paper analyzes the trends in the development of camera technology, and designs a color night vision camera.

最后根据市场情况和市面上产品的情况分析了摄像机技术的发展趋势,并设计了一款彩色夜视摄像机。

Only person height weeds and the fierce looks stone idles were there.

只有半人深的荒草和龇牙咧嘴的神像。

This dramatic range, steeper than the Himalayas, is the upturned rim of the eastern edge of Tibet, a plateau that has risen to 5 km in response to the slow but un stoppable collision of India with Asia that began about 55 million years ago and which continues unabated today.

这一引人注目的地域范围,比喜马拉雅山更加陡峭,是处于西藏东部边缘的朝上翻的边框地带。响应启始于约5500万年前的、缓慢的但却不可阻挡的印度与亚洲地壳板块碰撞,高原已上升至五千米,这种碰撞持续至今,毫无衰退。