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科学美国人60秒 卡夫利奖颁发:理解神经发育和神经退行性变

时间:2022-08-24 02:31:03

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(单词翻译)

The Kavli Prize Presents: Understanding Neurodevelopment and Neurodegeneration

卡夫利奖颁发:理解神经发育和神经退行性变

This podcast was produced for The Kavli Prize by Scientific American Custom Media, a division separate from the magazine’s board of editors.

这篇播客是由《科学美国》定制媒体为卡夫利奖制作的,该部门独立于该杂志的编辑委员会。

Megan Hall: Why would a normally developing girl stop walking?  What causes a middle-aged2 person to lose their sense of balance? Dr. Huda Zoghbi has devoted3 her career to unraveling these puzzles.

梅根·霍尔:为什么一个正常发育的女孩会停止走路?是什么导致中年人失去平衡感?胡达·佐格比博士的职业生涯致力于解开这些谜团。

She shares The Kavli Prize with Jean-Louis Mandel, Harry5 Orr, and Christopher Walsh for discovering the genetic6 pathways behind serious brain disorders8.

她与Jean-LouisMandel、HarryOrr和ChristopherWalsh共同获得卡夫利奖,因为他们发现了严重脑部疾病背后的遗传途径。

Scientific American Custom Media, in partnership9 with The Kavli Prize, spoke10 with Huda to learn more about her research.

美国科学定制媒体与卡夫利奖合作,与胡达交谈,以了解更多关于她的研究。

The Kavli Prize is a prestigious11 honor on its own, but the award holds a special place in Dr. Huda Zogbhi’s heart.

卡夫利奖本身就是一项享有盛誉的荣誉,但该奖在胡达·佐格比博士心中占有特殊地位。

Huda Zoghbi: Because it recognizes work that I have so cherished. It is work with my longtime collaborator12, Harry Orr, and I so cherish the work as well as our relationship over the years. So to me, this was the sweetest way to recognize that work.

胡达·佐格比:因为它认可了我非常珍惜的工作。这是与我的长期合作伙伴哈里·奥尔(HarryOrr)的合作,我非常珍惜这项工作以及我们多年来的关系。所以对我来说,这是认可这项工作的最甜蜜的方式。

Hall: Huda and Professor Harry Orr both received the Kavli Prize this year, for research that has been intertwined for decades.

霍尔:胡达和哈里·奥尔教授都获得了今年的卡夫利奖,因为他们的研究已经交织了几十年。

Zoghbi: Our collaboration13 has outlasted14 most American marriages.

佐比:我们的合作比大多数美国婚姻都要长久。

Hall: It all started when Huda was at the very beginning of her career. She was trying to unravel4 the genetic cause of a disorder7 that affected15 the balance and speech of a large family in Texas. At around 40 years old, affected family members ...

霍尔:这一切都始于胡达职业生涯刚开始的时候。她试图解开一种疾病的遗传原因,这种疾病影响了德克萨斯州一个大家庭的平衡和语言。在40岁左右,受影响的家庭成员

Zoghbi: ... will start feeling a little bit off-balance, if they're making a quick move. And slowly their speech becomes slurred16. And that gets worse and worse with time.

佐比:。。。如果他们快速移动,会开始感觉有点失衡。慢慢地,他们的言语变得含糊不清。随着时间的推移,情况越来越糟

Hall: Eventually, the family members lose their ability to walk and talk clearly. They typically die around 20 years later, of causes related to breathing or swallowing problems.

The disorder is known as spino-cerebellar ataxia type 1, or SCA-1.

霍尔:最终,家庭成员失去了清晰行走和说话的能力。他们通常在20年后死于呼吸或吞咽问题。

这种疾病被称为脊髓-小脑共济失调1型,或SCA-1。

Zoghbi: It’s a family with 200 members, and I started immediately driving every few days to Montgomery and collecting samples.

佐比:这是一个有200人的家庭,我开始每隔几天就开车去蒙哥马利采集样本。

Hall: With the samples Huda gathered and the help of her colleagues, she discovered that the gene1 responsible for SCA-1 was located on chromosome17 6. But, she still had a long way to go.

霍尔:在胡达收集的样本和同事的帮助下,她发现负责SCA-1的基因位于6号染色体上。但是,她还有很长的路要走。

Zoghbi: Imagine you map it to the state of Texas, right? And now you want to find where the house is. So we have to really get the map closer and closer and narrow in to get close to it.

Hall: Time passed, and she finally started getting closer to the location of the gene. Let’s say, she’d located the city.

佐比:想象一下你把它映射到德克萨斯州,对吗?现在你想知道房子在哪里。所以我们必须让地图越来越近,越来越窄,才能接近它。

霍尔:随着时间的推移,她终于开始接近基因的位置。比如说,她找到了这个城市。

She also discovered that Professor Harry Orr at the University of Minnesota was studying a similar disorder in the same general area of chromosome 6.

Zoghbi: I read papers by him showing that we are in the same city. And I was like, wow, this guy is impressive. He's done all this work.

她还发现明尼苏达大学的哈里·奥尔教授正在研究6号染色体相同区域的类似疾病。

佐比:我看了他写的文件,表明我们在同一个城市。我想,哇,这家伙真让人印象深刻。他完成了所有这些工作。

Hall: They eventually met and started sharing information. But, over time, it became clear that they were looking at genes18 in different locations.

Zoghbi: By then Harry had an inkling that his gene is towards one side, let's say the northern side of the city. And I had data to suggest it's at the southern side of the city. So we're far apart.

霍尔:他们最终会面并开始分享信息。但是,随着时间的推移,很明显他们正在研究不同位置的基因。

佐比:到那时,哈利已经知道他的基因是朝着一边的,比如说城市的北边。我有数据表明它在城市的南边。所以我们相距遥远。

Hall: Huda learned a complicated technique to create little “addresses” or markers on chromosome 6 to better locate her gene. And she thought, why not share them with Harry?

Zoghbi: So I called him up. And I said, look, I made those hybrids19. If you want to use them, please go ahead and take them and use them. And he was like, great. So, we’re communicating, we’re having a really beautiful, cordial relationship.

霍尔:胡达学会了一种复杂的技术,在6号染色体上创建小“地址”或标记,以便更好地定位她的基因。她想,为什么不和哈利分享呢?

佐比:所以我打电话给他。我说,看,我做了那些混合动力车。如果你想使用它们,请继续使用它们。他说,太好了。所以,我们在交流,我们有一个非常美丽、亲切的关系。

Hall: Huda went on with her research, but she had this nagging20 thought in the back of her brain.

Zoghbi: I think there's something fishy21 here.

Hall: How could it be that she and Harry were studying two different diseases if both had similar symptoms, with a genetic cause in the same general region of chromosome 6?

霍尔:胡达继续她的研究,但她脑子里有一个烦人的想法。

佐比:我觉得这里有点可疑。

霍尔:如果她和哈里都有相似的症状,遗传原因在6号染色体的同一个一般区域,那怎么可能她和哈里在研究两种不同的疾病呢?

Zoghbi: I kept pushing and pushing.

Hall: Eventually, Huda found a mistake in the data from the family she was studying—everyone had assumed a group of daughters had inherited SCA-1 from their mother. But it turns out, it was actually the father who passed the disorder to his girls.

佐比:我一直推啊推。

霍尔:最终,胡达在她所研究的家庭数据中发现了一个错误,每个人都认为有一群女儿从母亲那里继承了SCA-1。但事实证明,是父亲把疾病传给了他的女儿们。

Huda quickly worked with a technician to rerun some of her experiments.

Zoghbi: Cataloging everything for all the branches of this family to construct what came from dad, what came from mom. And when we did that, it fell on top of Harry's gene.

Hall: She immediately called Harry.

胡达很快与一名技术人员合作,重新进行了一些实验。

Zoghbi:为这个家族的所有分支编目,以构建什么来自爸爸,什么来自妈妈。当我们这样做的时候,它落在了哈利的基因上。

霍尔:她立刻打电话给哈利。

Zoghbi: I said, Harry, I use this marker, it puts it right on top of your gene. We're working on the same disease.

Hall: Huda was relieved, but Harry was worried.

Zoghbi: Because back then cloning a disease gene was a big deal. Everybody wanted the glory to themselves.

佐比:我说,哈利,我用这个标记,它把它放在你基因的正上方。我们正在研究同一种疾病。

胡达松了口气,但哈利很担心。

佐比:因为当时克隆疾病基因是一件大事。每个人都想把荣耀留给自己。

Hall: Harry asked if Huda wanted him to return the resources she’d shared with him.

Zoghbi: I said, no, no, no, no, you keep them. We work together. Now we really collaborate22. Now we're working on the same thing.

15 seconds of silence. And he said, Let's do it.

霍尔:哈里问胡达是否希望他归还她与他共享的资源。

佐比:我说,不,不,不,不,你留着。我们一起工作。现在我们真的合作了。现在我们正在做同样的事情。

15秒的沉默。他说,我们来做吧。

Hall: When Huda and Harry combined their data, they were able to narrow the location of their gene down to about one million base pairs. They slowly examined each gene one at a time.

But then, Huda heard a scientific talk about a disorder that was marked by repeating letters of DNA23.

霍尔:当胡达和哈里结合他们的数据时,他们能够将基因的位置缩小到大约一百万个碱基对。他们一次一个地慢慢检查每个基因。

但后来,胡达听到了一个关于一种以重复DNA字母为特征的疾病的科学讨论。

Zoghbi: I said, Harry, we're not going to walk gene by gene. I just heard this awesome24 talk from Tom Caskey and it's these three bases of DNA that repeat.  Let's just ignore everything else and focus on finding repeats.

佐比:我说,哈利,我们不会一个基因一个基因走。我刚刚听到汤姆·卡斯基(TomCaskey)的精彩演讲,正是这三个DNA碱基在重复。让我们忽略其他一切,专注于寻找重复。

Hall: So, she and Harry divided their genes up,  including a little bit of overlap25, and started hunting.

A few weeks later, on the same day...

霍尔:所以,她和哈里把他们的基因分了开来,包括一点重叠,然后开始打猎。

几周后的同一天。。。

Zoghbi: April 8, 1993. He sent me a fax, he discovered the mutation26 in his family and I sent him a fax, I discovered the mutation in my family.

Hall: Huda and Harry have been working together ever since. Before Huda submits a grant proposal, she always lets Harry know.

佐比:1993年4月8日。他给我发了一份传真,他在家里发现了突变,我给他发了一份传真,我在家里发现了突变。

霍尔:胡达和哈里一直在一起工作。在胡达提交拨款申请之前,她总是让哈利知道。

Zoghbi: Harry, I'm going to do this, this, and this. And he goes perfect. I'm not doing any of that. I will write a letter to assure the reviewers and to tell them how I will help you. And I do the same for him.

佐比:哈利,我要做这个,这个,还有这个。他很完美。我什么都没做。我将写一封信向评审人员保证,并告诉他们我将如何帮助您。我也为他做了同样的事。

Hall: Between them, they’ve discovered that the gene responsible for SCA-1 produces a protein called ATAXIN-1 that causes clumps27 in the brain and leads to that loss of balance.

They’ve also developed a new type of treatment that improves SCA-1 symptoms in mice.

It’s a rare collaboration in a world that’s highly competitive. What made them do it?

霍尔:在他们之间,他们发现负责SCA-1的基因会产生一种叫做共济失调蛋白-1的蛋白质,这种蛋白质会导致大脑结块并导致平衡的丧失。

他们还开发了一种新型治疗方法,可以改善小鼠的SCA-1症状。

在一个竞争激烈的世界里,这是一次罕见的合作。是什么让他们这么做的?

Zoghbi: I don't think either of us really thought about who's gonna get credit. Honestly. We just wanted to solve this problem. And I think that was the driver.

Hall: Also, they were young.

Zoghbi: I would say most of my good decisions were due to naivety28. You know, trust your heart and don’t overthink it, really.

佐比:我认为我们两人都没有真正想过谁会得到信贷。真的我们只是想解决这个问题。我想那就是司机。

霍尔:而且,他们还年轻。

佐比:我想说,我的大多数好决定都是由于天真。你知道,相信你的心,不要想得太多,真的。

Hall: Huda says this collaboration has deepened their knowledge not just of SCA-1, but other neurological disorders, like Alzheimer's and Parkinson's disease.

Zoghbi: In my long-term vision is prevention. And I'm excited about venturing in that area and finding things that we can maybe take a small pill for that’s safe for people who had a family history of disease or had an at-risk genotype.

霍尔:胡达说,这项合作不仅加深了他们对SCA-1的了解,而且加深了他们对其他神经系统疾病的了解,如阿尔茨海默病和帕金森病。

佐比:我的长期愿景是预防。我很高兴能在这一领域冒险,找到一些我们可以服用的小药丸,对有家族病史或有危险基因型的人来说是安全的。

Hall: Her work with Harry is already being used in clinical trials for treating SCA-1 and other disorders.  Her advice for scientists that want to follow in her path? Be patient.

Zoghbi: Everybody looks at me and gets excited because of the big discovery. I want to remind everybody there were years for each of these discoveries. And that's okay.

霍尔:她与哈里的合作已经用于治疗SCA-1和其他疾病的临床试验。她给那些想追随她的科学家们的建议?要有耐心。

佐比:每个人都看着我,因为这个重大发现而感到兴奋。我想提醒大家,这些发现都有好几年的时间。没关系。

Hall: Huda says, in science, it might take a long time for success to come. But when it does, it’s so satisfying. Especially when it’s shared with a good friend.

Dr. Huda Zoghbi is a Professor at Baylor College of Medicine, the Director of the Jan and Dan Duncan Neurological Research Institute at Texas Children’s Hospital and an Investigator29 with the Howard Hughes Medical Institute.

霍尔:胡达说,在科学领域,成功可能需要很长时间。但当它出现时,它是如此令人满意。尤其是当它与一个好朋友分享时。

Huda Zoghbi博士是贝勒医学院的教授,德克萨斯州儿童医院Jan和Dan Duncan神经研究所所长,霍华德·休斯医学研究所的研究员。

This year, she shared The Kavli Prize in Neuroscience with Harry Orr, Jean-Louis Mandel, and Christopher Walsh.

今年,她与哈里·奥尔、让·路易斯·曼德尔和克里斯托弗·沃尔什共同获得了卡夫利神经科学奖。

The Kavli Prize honors scientists for breakthroughs in astrophysics, nanoscience, and neuroscience—transforming our understanding of the big, the small and the complex. The Kavli Prize is a partnership among the Norwegian Academy of Science and Letters, The Norwegian Ministry30 of Education and Research, and the US-based Kavli Foundation.

卡夫利奖表彰在天体物理学、纳米科学和神经科学方面取得突破的科学家,这些突破改变了我们对大、小和复杂的理解。卡夫利奖是挪威科学与文学学院、挪威教育与研究部和美国卡夫利基金会之间的合作伙伴。

This work was produced by Scientific American Custom Media and made possible through the support of The Kavli Prize.

该作品由美国科学定制媒体制作,并通过卡夫利奖的支持得以实现。


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点击收听单词发音收听单词发音  

1 gene WgKxx     
n.遗传因子,基因
参考例句:
  • A single gene may have many effects.单一基因可能具有很多种效应。
  • The targeting of gene therapy has been paid close attention.其中基因治疗的靶向性是值得密切关注的问题之一。
2 middle-aged UopzSS     
adj.中年的
参考例句:
  • I noticed two middle-aged passengers.我注意到两个中年乘客。
  • The new skin balm was welcome by middle-aged women.这种新护肤香膏受到了中年妇女的欢迎。
3 devoted xu9zka     
adj.忠诚的,忠实的,热心的,献身于...的
参考例句:
  • He devoted his life to the educational cause of the motherland.他为祖国的教育事业贡献了一生。
  • We devoted a lengthy and full discussion to this topic.我们对这个题目进行了长时间的充分讨论。
4 unravel Ajzwo     
v.弄清楚(秘密);拆开,解开,松开
参考例句:
  • He was good with his hands and could unravel a knot or untangle yarn that others wouldn't even attempt.他的手很灵巧,其他人甚至都不敢尝试的一些难解的绳结或缠在一起的纱线,他都能解开。
  • This is the attitude that led him to unravel a mystery that long puzzled Chinese historians.正是这种态度使他解决了长期以来使中国历史学家们大惑不解的谜。
5 harry heBxS     
vt.掠夺,蹂躏,使苦恼
参考例句:
  • Today,people feel more hurried and harried.今天,人们感到更加忙碌和苦恼。
  • Obama harried business by Healthcare Reform plan.奥巴马用医改掠夺了商界。
6 genetic PgIxp     
adj.遗传的,遗传学的
参考例句:
  • It's very difficult to treat genetic diseases.遗传性疾病治疗起来很困难。
  • Each daughter cell can receive a full complement of the genetic information.每个子细胞可以收到遗传信息的一个完全补偿物。
7 disorder Et1x4     
n.紊乱,混乱;骚动,骚乱;疾病,失调
参考例句:
  • When returning back,he discovered the room to be in disorder.回家后,他发现屋子里乱七八糟。
  • It contained a vast number of letters in great disorder.里面七零八落地装着许多信件。
8 disorders 6e49dcafe3638183c823d3aa5b12b010     
n.混乱( disorder的名词复数 );凌乱;骚乱;(身心、机能)失调
参考例句:
  • Reports of anorexia and other eating disorders are on the increase. 据报告,厌食症和其他饮食方面的功能紊乱发生率正在不断增长。 来自《简明英汉词典》
  • The announcement led to violent civil disorders. 这项宣布引起剧烈的骚乱。 来自《简明英汉词典》
9 partnership NmfzPy     
n.合作关系,伙伴关系
参考例句:
  • The company has gone into partnership with Swiss Bank Corporation.这家公司已经和瑞士银行公司建立合作关系。
  • Martin has taken him into general partnership in his company.马丁已让他成为公司的普通合伙人。
10 spoke XryyC     
n.(车轮的)辐条;轮辐;破坏某人的计划;阻挠某人的行动 v.讲,谈(speak的过去式);说;演说;从某种观点来说
参考例句:
  • They sourced the spoke nuts from our company.他们的轮辐螺帽是从我们公司获得的。
  • The spokes of a wheel are the bars that connect the outer ring to the centre.辐条是轮子上连接外圈与中心的条棒。
11 prestigious nQ2xn     
adj.有威望的,有声望的,受尊敬的
参考例句:
  • The young man graduated from a prestigious university.这个年轻人毕业于一所名牌大学。
  • You may even join a prestigious magazine as a contributing editor.甚至可能会加入一个知名杂志做编辑。
12 collaborator gw3zSz     
n.合作者,协作者
参考例句:
  • I need a collaborator to help me. 我需要个人跟我合作,帮我的忙。
  • His collaborator, Hooke, was of a different opinion. 他的合作者霍克持有不同的看法。
13 collaboration bW7yD     
n.合作,协作;勾结
参考例句:
  • The two companies are working in close collaboration each other.这两家公司密切合作。
  • He was shot for collaboration with the enemy.他因通敌而被枪毙了。
14 outlasted 0c30f8ec77eacb5d664fb2516a1b072b     
v.比…长久,比…活得长( outlast的过去式和过去分词 )
参考例句:
  • I outlasted several downsizings but the last one included me. 虽然我坚持到了最后,还是逃不过被裁的命运。 来自互联网
  • This clock has outlasted several owners. 这座时钟的寿命比它的几个主人的寿命都长。 来自互联网
15 affected TzUzg0     
adj.不自然的,假装的
参考例句:
  • She showed an affected interest in our subject.她假装对我们的课题感到兴趣。
  • His manners are affected.他的态度不自然。
16 slurred 01a941e4c7d84b2a714a07ccb7ad1430     
含糊地说出( slur的过去式和过去分词 ); 含糊地发…的声; 侮辱; 连唱
参考例句:
  • She had drunk too much and her speech was slurred. 她喝得太多了,话都说不利索了。
  • You could tell from his slurred speech that he was drunk. 从他那含糊不清的话语中你就知道他喝醉了。
17 chromosome 7rUzX     
n.染色体
参考例句:
  • Chromosome material with exhibits of such behaviour is called heterochromatin.表现这种现象的染色体物质叫做异染色质。
  • A segment of the chromosome may become lost,resulting in a deletion.染色体的一个片段可能会丢失,结果产生染色体的缺失。
18 genes 01914f8eac35d7e14afa065217edd8c0     
n.基因( gene的名词复数 )
参考例句:
  • You have good genes from your parents, so you should live a long time. 你从父母那儿获得优良的基因,所以能够活得很长。 来自《简明英汉词典》
  • Differences will help to reveal the functions of the genes. 它们间的差异将会帮助我们揭开基因多种功能。 来自英汉非文学 - 生命科学 - 生物技术的世纪
19 hybrids a5030918be299fefcf603b9326766b39     
n.杂交生成的生物体( hybrid的名词复数 );杂交植物(或动物);杂种;(不同事物的)混合物
参考例句:
  • All these brightly coloured hybrids are so lovely in the garden. 花园里所有这些色彩鲜艳的杂交花真美丽。 来自辞典例句
  • The notion that interspecific hybrids are rare is ill-founded. 有一种看法认为种间杂种是罕见的,这种看法是无根据的。 来自辞典例句
20 nagging be0b69d13a0baed63cc899dc05b36d80     
adj.唠叨的,挑剔的;使人不得安宁的v.不断地挑剔或批评(某人)( nag的现在分词 );不断地烦扰或伤害(某人);无休止地抱怨;不断指责
参考例句:
  • Stop nagging—I'll do it as soon as I can. 别唠叨了—我会尽快做的。
  • I've got a nagging pain in my lower back. 我后背下方老是疼。 来自《简明英汉词典》
21 fishy ysgzzF     
adj. 值得怀疑的
参考例句:
  • It all sounds very fishy to me.所有这些在我听起来都很可疑。
  • There was definitely something fishy going on.肯定当时有可疑的事情在进行中。
22 collaborate SWgyC     
vi.协作,合作;协调
参考例句:
  • The work gets done more quickly when we collaborate.我们一旦合作,工作做起来就更快了。
  • I would ask you to collaborate with us in this work.我们愿意请你们在这项工作中和我们合作。
23 DNA 4u3z1l     
(缩)deoxyribonucleic acid 脱氧核糖核酸
参考例句:
  • DNA is stored in the nucleus of a cell.脱氧核糖核酸储存于细胞的细胞核里。
  • Gene mutations are alterations in the DNA code.基因突变是指DNA密码的改变。
24 awesome CyCzdV     
adj.令人惊叹的,难得吓人的,很好的
参考例句:
  • The church in Ireland has always exercised an awesome power.爱尔兰的教堂一直掌握着令人敬畏的权力。
  • That new white convertible is totally awesome.那辆新的白色折篷汽车简直棒极了.
25 overlap tKixw     
v.重叠,与…交叠;n.重叠
参考例句:
  • The overlap between the jacket and the trousers is not good.夹克和裤子重叠的部分不好看。
  • Tiles overlap each other.屋瓦相互叠盖。
26 mutation t1PyM     
n.变化,变异,转变
参考例句:
  • People who have this mutation need less sleep than others.有这种突变的人需要的睡眠比其他人少。
  • So far the discussion has centered entirely around mutation in the strict sense.到目前为止,严格来讲,讨论完全集中于围绕突变问题上。
27 clumps a9a186997b6161c6394b07405cf2f2aa     
n.(树、灌木、植物等的)丛、簇( clump的名词复数 );(土、泥等)团;块;笨重的脚步声v.(树、灌木、植物等的)丛、簇( clump的第三人称单数 );(土、泥等)团;块;笨重的脚步声
参考例句:
  • These plants quickly form dense clumps. 这些植物很快形成了浓密的树丛。 来自《简明英汉词典》
  • The bulbs were over. All that remained of them were clumps of brown leaves. 这些鳞茎死了,剩下的只是一丛丛的黃叶子。 来自《简明英汉词典》
28 naivety 0FLxO     
n.天真,纯朴,幼稚
参考例句:
  • Mozart's music is characterized by its naivety and clarity.莫扎特的音乐特色是纯朴兴清澈。
  • She has lost none of her naivety.她丝毫没有失去那份天真烂漫。
29 investigator zRQzo     
n.研究者,调查者,审查者
参考例句:
  • He was a special investigator for the FBI.他是联邦调查局的特别调查员。
  • The investigator was able to deduce the crime and find the criminal.调查者能够推出犯罪过程并锁定罪犯。
30 ministry kD5x2     
n.(政府的)部;牧师
参考例句:
  • They sent a deputation to the ministry to complain.他们派了一个代表团到部里投诉。
  • We probed the Air Ministry statements.我们调查了空军部的记录。

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