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本发明涉及一种治疗杜兴氏肌营养不良疾病的药物。The present invention is one medicine for treating Duchenne muscular dystrophy.
有趣的是只有十分之一的人,可以伪装真正的微笑。What's interesting is about only one out of every ten people can fake a Duchenne smile.
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杜氏肌萎缩症是一种致死性的基因突变疾病,平均每3500个男孩中就有一个罹患此病。Duchenne Muscular Dystrophy is a fatal genetic mutation in about one of every 3,500 boys.
研究者将这些照片分为没有微笑、杜乡微笑和非杜乡微笑三类。The researchers classified them as non-smilers, Duchenne smilers or non-Duchenne smilers.
他最近利用此法治疗动物的杜兴氏肌营养不良症,结果很有效。He recently used it to treat animals with Duchenne muscular dystrophy, and the results have been promising.
他们发现在去世的球手中,杜乡微笑的球手往往最长寿,其次是非杜乡微笑的球手。They found that out of the dead players, Duchenne smilers had tended to live the longest, followed by non-Duchenne smilers.
真正开心的微笑是杜乡式微笑,在一个神经心理学者研究后发现,包括眨眼睛。A real happiness smile, what's known as a Duchenne smile, after a neurophysiologist who studied it, involves moving the eyes.
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被称为PTC124的药物,已经在治疗Duchenne肌营养不良和囊性纤维化的病人中获得了激动人心的成果。The drug, known as PTC124, has already had encouraging results in patients with Duchenne muscular dystrophy and cystic fibrosis.
插入可导致很多遗传病,如血友病、杜氏肌营养不良,并且还可能与肿瘤的发展有关。Insertions can cause many genetic diseases, such as hemophilia and Duchenne muscular dystrophy, and may play a role in the development of cancer.
类似地,杜兴认为真实的笑容需要牵动眼部周围的轮匝肌,而这是无法有意控制的。Similarly, Duchenne identified a genuine smile as using the orbicularis oculi muscle around the eyes which cannot easily be consciously manipulated.
目的在18个缺失型迪谢内及贝克肌营养不良家系中,比较有效检测基因携带者的方法。Objective To develop and compare the methods for determining the carrier status in the 18 deleted families of Duchenne and Becker muscular dystrophy.
由于后者的肌肉使动的表情是很自然的,所以,像鸟足一样弯成扇形的微笑,是被认作为最自然、最真实的表情,也就是众所周知的真真正正微笑。Now it’s this latter muscle that’s involuntary, so the crows feet smile is considered the real spontaneous emotion and is known as the Duchenne smile.
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比如,在Duchenne肌肉营养不良中,正常肌肉发育的必须蛋白没有被合成,其结果就是致死性萎缩疾病。In Duchenne muscular dystrophy, for example, the protein necessary for normal muscle development is not made, and the fatal wasting disease is the result.
“这是用第一个用干细胞治疗的方法来治疗杜氏肌萎缩的成功方法,”他说。"This is the first piece of research that has convinced me that stem cell therapy could play a role in treatment for Duchenne muscular dystrophy, " he said.
对于你朋友的孩子,有个基因导致杜氏抗肌萎缩蛋白缺失,而目前的治疗并不能对这个基因有所作用。As for your friend's children, they have a mutation causing their Duchenne muscular dystrophy that will not be helped by the drug we are currently trialling.
这个实验极有可能改善千千万万杜氏肌肉萎缩症患儿的生活。With the efficacy of biglycan apparent in the mouse model of Duchenne Muscular Dystrophy, Fallon is eager to see if it can improve the lives of thousands of children.
因而纤维化被认为是肌肉组织破坏后之修复反应。但是大臀肌枸缩症与裘馨型进行性肌肉萎缩症有较严重之纤维变化。The above changes varied in degree among different kinds of myopathies, but the Duchenne muscular dystrophy and gluteal fibrosis groups were the most severely affected.
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杜兴是一名神经病学家,他对相貌如何产生面部表情感兴趣,并认为那与人的灵魂有直接关系。Duchenne was a French neurologist interested in determining how the physiognomy of the face produced facial expressions, which he thought were directly linked to the soul.
他们发现,在年鉴照片杀回来过表现出杜乡微笑的职业棒球运动员,在任何一年,死亡几率只有其他没有表现出杜乡微笑运动员的一半。They found that professional baseball players who sported Duchenne smiles in their yearbook photo were only half as likely to die, in any given year, as those who had not.
对后代属于罕见性别相关遗传疾病的高危人群的父母来说,这可能是一个好消息,比如男孩的Duchenne肌肉萎缩症和女孩的Turner综合症。This could be good news for parents whose offspring are at risk of rare gender-related genetic disorders, like Duchenne muscular dystrophy in boys or Turner syndrome in girls.