纪录片部落--纪录片《[BBC纪录片]试错TrialandError-1080P高清迅雷网盘下载》高清百度云1080p下载
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：试错Trial and Error-1080P高清迅雷网盘下载
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由Dilly Barlow主持并由???BBC发行的科学纪录片，作为2003年BBC Horizo??n系列的一部分播出-英语旁白Science Documentary hosted by Dilly Barlow and published by BBC broadcasted as part of BBC Horizon series in 2003 - English narration
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这是最简单的想法，但潜力巨大。如果一个基因在人体中有缺陷，只需将其替换为一个可以正常工作的基因即可。基因疗法将意味着遗传疾病将成为过去。癌症将得以治愈，囊性纤维化和数百种其他遗传疾病也会被治愈。科学家对这个想法感到很兴奋，但是这种热情最终使一个年轻人丧命。杰西·格辛格（Jesse Gelsinger）出生时患有肝脏疾病，一种罕见的病症，称为鸟氨酸转氨甲酰酶（OTC）缺乏症，会阻止肝脏代谢氨。患有这种疾病的人可能会遭受脑部损伤或昏迷。在最极端的情况下，这种疾病是致命的。“ 1998年，杰西（Jesse）像我以前所认识的一样健康。”杰西（Jesse）的父亲保罗·格辛格（Paul Gelsinger）尽管他每天喝一些毒品来控制自己的病情，但他过着相当正常的生活。杰西想帮助别人。当他有机会参加医学试验以测试使用基因疗法治疗OTC缺乏症的安全性时，他渴望参加。他知道这不是治愈他的病的方法，而是通过自愿他将来可能会帮助别人。提供治疗方法尽管基因疗法的概念很简单，但是治疗方法的实践却要困难得多。为了替换有缺陷的基因，医生必须让工作基因进入人体并进入需要它们的地方。科学家们巧妙地解决了这个问题。劫持我们最致命的敌人之一-病毒。病毒通过将有害基因直接插入细胞内来感染人类。通常这会对细胞造成损害，使我们生病，但科学家们坚信他们可以驯服这种自然能力。用好的基因代替有害的基因，该病毒将是完美的传递载体或载体。最初认为逆转录病毒是将修饰的基因带入体内的最佳方法。大多数病毒和细胞生物将其遗传物质存储为脱氧核糖核酸（DNA）。逆转录病毒改为使用核糖核酸（RNA）。逆转录病毒将其遗传物质与宿主的遗传物质永久结合在一起，因此它们可以提供永久性治愈。不幸的是，使用逆转录病毒进行的试验无法成功治愈除一种特定类型的血液病，严重的联合免疫缺陷病（SCID）以外的任何疾病。英国医生最近使用这种技术治愈了五岁的Rhys Evans，并取得了巨大的成功。科学家意识到，如果要治疗心脏，肝脏或肺等人体器官，他们将不得不寻找另一种方式进行治疗-逆转录病毒根本无法进入。答案将来自受人尊敬的科学家詹姆斯博士威尔逊（Wilson）于1992年成立了世界上最大的基因治疗中心。他建议使用最常见的病毒：腺病毒，引起普通感冒。腺病毒似乎是一个不错的选择，因为它能够感染人体的几乎所有细胞。威尔逊试验需要一种疾病来试验他的腺病毒载体，并偶然将目光投向了影响杰西·格辛格（Jesse Gelsinger）的OTC缺乏症。因此，在1998年9月，杰西和他的家人被要求参加审判。杰西知道参加比赛不会治愈他的非处方药，但他热衷于提供帮助，因为他知道如果成功It was the simplest idea but one with enormous potential. If a gene is defective in the human body, just replace it with one that works properly. Gene therapy would mean that genetic disorders would become a thing of the past. Cancer would be cured, as would cystic fibrosis and hundreds of other genetic illnesses. Scientists were justifiably excited about the idea but, this enthusiasm that would end up costing one young man his life.Jesse Gelsinger was born with a liver disorder, a rare condition called ornithine transcarbamylase (OTC) deficiency that stops the liver metabolising ammonia. People with the disease can suffer from brain damage or coma. At its most extreme the illness is fatal. "In 1998, Jesse was as healthy as I had ever known him" Paul Gelsinger, Jesse's fatherJesse was lucky, able to lead a fairly normal life although he had a daily cocktail of drugs to control his condition. Jesse wanted to help others. When he was offered a chance to take part in a medical trial to test the safety of using gene therapy for OTC deficiency, he was keen to participate. He knew this was not a cure for his condition but that, by volunteering he might be able to help others in the future.Delivering a cureAlthough the concept of gene therapy is simple, the practice of administering the treatment is much more difficult. In order to replace defective genes, doctors must get working ones into the body and to the place where they are needed.Scientists had an ingenious solution to the problem. Hijack one of our most deadly enemies - the virus. A virus infects a human by inserting its harmful genes directly inside our cells. Normally this causes damage to the cell making us sick, but scientists were convinced they could tame this natural ability. Replace the harmful genes with good ones, and the virus would be the perfect delivery vehicle, or vector.It was initially believed that a retrovirus would be the best way of getting modified genes into the body. Most viruses and cellular organisms store their genetic material as deoxyribonucleic acid (DNA). Retroviruses use ribonucleic acid (RNA) instead. Retroviruses combine their genetic material with that of the host permanently, hence they can offer a permanent cure.Unfortunately trials using retroviruses had very little success treating any illness other than one particular type of blood disorder, severe combined immunodeficiency (SCID). British doctors have recently used this technique to cure five year old Rhys Evans with great success. Scientists realised that if they wanted to treat organs in the body like the heart, liver or lungs, they would have to find an alternative way of delivering their treatment - retroviruses simply couldn't get in.The answer would come from respected scientist Dr James Wilson, who had in 1992 set up the largest gene therapy centre in the world. He proposed using the most common virus around: the adenovirus, cause of the common cold. The adenovirus seemed like a good choice because it is able to affect almost every cell in the human body.The trialWilson needed a disease to trial his adenovirus vector on, and by chance set his sights on OTC deficiency, the disease affecting Jesse Gelsinger. So in September 1998 Jesse and his family were approached to participate in a trial. Jesse knew that taking part was not going to cure his OTC but he was keen to help because he knew that if successful he would have played a part in curing thousands of diseases.On 12 September 1999 Jesse arrived at the University of Pennsylvania to begin the trial. One day later a member of Wilson's team injected Jesse with the tame viral particles. He was given the biggest adenovirus dose of any of the trial participants. The doctors told Jesse to expect a small reaction to the virus.On the 14th when a nurse checked on Jesse she found that he was slightly confused and jaundiced. Although they doubted this was serious, the team wanted to be sure. The adenovirus was supposed to be harmless but Jesse's body was behaving like it was under attack. By the next day Jesse was in a coma.Over the next two days Jesse's condition deteriorated until on day five, Wilson's team of doctors delivered the devastating news to his parents. Jesse had no brain activity, his internal organs were shutting down. The doctors suggested that Jesse be removed from life support. Jesse died at 2.30 pm on 17 September.Learning the lessonsIn the aftermath of Jesse's death, it emerged that vital facts about the dangers of the vector had never been given to the Gelsingers. What chance of success is there now for gene therapy and at what risk? For many people the dream of gene therapy is dead. It is not the cure all that we dreamed of ten years ago but instead it is a highly selective treatment for a very small number of diseases. Gene therapy may still become an effective treatment for thousands of people but this is no miracle cure.
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【技术参数】——
视频编码: Divx 5.21
比特率: 1882 kb/s
Video 分辨率: 688x512 (1.34:1)
Video 画面比例: 4:3
音频编码: MP3
音频比特率: 130 kb/s (65/ch) VBR 48000 Hz
分集时长: 48m 26s
体积: 700mb
发布人： jvt40【Technical Specs】——
Video Codec: Divx 5.21
Video Bitrate: 1882 kb/s
Video Resolution: 688x512 (1.34:1)
Video Aspect Ratio: 4:3
Audio Codec: MP3
Audio BitRate: 130 kb/s (65/ch) VBR 48000 Hz
RunTime: 48m 26s
Part Size: 700mb
Ripped by jvt40
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