UT Southwestern Medical Center - Tetrabenazine, a drug used in some countries to treat the symptoms of Huntington’s disease, prevents death of brain cells in mice genetically engineered to mimic the hereditary condition, researchers have found.德州大学医学中心-嗪，药物使用，一些国家对待症状亨廷顿氏病，防止死亡的脑细胞在鼠的基因工程，以模仿世袭条件下，研究人员已经发现。

The research sheds light on the biochemical mechanisms involved in the disease and suggests new avenues of study for preventing brain-cell death in at-risk people before symptoms appear.研究揭示的生化机制所涉及的疾病，并提出了新的途径的研究，为防止脑细胞死亡，在高危人群中，才出现症状。

“The drug can actually prevent brain cells from dying,” said Dr. Ilya Bezprozvanny, associate professor of physiology at UT Southwestern. "毒品其实可以防止脑细胞死亡，博士说： "伊利亚bezprozvanny ，副教授生理学在德州大学的。 “It’s much more important than people thought.” "这是更为重要的，比人们想的" 。

The study, of which Dr. Bezprozvanny is senior author, appears in the July 25, 2007 issue of The Journal of Neuroscience .研究人，其中博士bezprozvanny是资深作家，出现在2007年7月25日发行的杂志上的神经系统 。

The drug, called tetrabenazine (TBZ), is commercially distributed as Xenazine or Nitoman and blocks the action of dopamine, a compound that some nerve cells use to signal others.该药物名为嗪（ tbz ） ，是商业分布xenazine或nitoman和盖帽的行动，多巴胺，复合型，有些神经细胞用信号等。 TBZ is approved for use in several countries, but not the U.S., to treat uncontrollable muscle movements in Huntington’s and other neurological conditions. tbz是批准使用在好几个国家，而不是美国，以治疗无法控制肌肉的运动，在亨廷顿的和其他神经系统的条件。

Huntington’s is a fatal genetic condition that usually manifests around ages 30 to 45, according to the Huntington’s Disease Society of America.亨廷顿的是一种致命的遗传条件，即通常舱单左右年龄30至45个，根据我的亨廷顿舞蹈病社会美利坚合众国。 About one in 10,000 people in America have the disease, with another 200,000 at risk.大约每1万人，在美国有这种疾病，另外有20万处于危险之中。 One of the most famous people with Huntington’s was folk singer Woody Guthrie, who died in 1967.其中最有名的人，与亨廷顿的是民歌歌手木本伽丝瑞，死于1967年。

Huntington’s is caused by a dominant gene, meaning that a person carrying the gene is certain to develop the disease and has a 50 percent chance of passing it on to his or her children.亨廷顿的，是由一种显性基因，即一个人携带这种基因，是注定要发展的疾病，并有百分之五十的机会，通过它，以他或她的孩子。 Symptoms include jerky, uncontrollable movements called chorea and deterioration of reasoning abilities and personality.症状包括干的，不可控的变动所谓舞蹈病和恶化的推理能力和个性。 Symptoms begin after many brain cells have already died.症状开始后，许多脑细胞已经死亡。

Although a genetic test exists, some people with a family history of Huntington’s choose not to be tested because there is no cure and because they fear loss of health insurance.虽然基因测试的存在，一些人对家族病史的亨廷顿的选择不被考验，因为目前尚缺乏有效的，因为他们害怕失去健康保险。 There are treatments to lessen the symptoms, but there is currently no way to slow or halt the progression of the disease.有治疗，以减轻症状，但有一点是目前还没有办法减缓或停止进展的疾病。

In the current study, the UT Southwestern researchers used mice that were genetically engineered to carry the mutant human gene for Huntington’s, causing symptoms and death of brain cells similar to those seen in the disease.在目前的研究中，德州大学的研究人员利用老鼠都经过基因工程改造，从而促使携带变异基因的人，为亨廷顿的，造成的症状和死亡的脑细胞相似，那些出现在疾病。 The study focused on an area of the brain called the striatum, which plays a critical role in relaying signals concerning motion and higher thought and receives signals from several brain regions.这项研究集中于一个地区的所谓大脑纹状体，其中起着关键作用，在转播信号的有关议案和更高的思想和接收信号的几个脑区。

The striatum is primarily made up of nerve cells called medium spiny neurons, which undergo widespread death in Huntington’s.纹状体主要是弥补了神经细胞的所谓中等刺神经元，其中进行广泛的死亡，在亨廷顿的。 One major input to the striatum comes from an area called the substantia nigra, which controls voluntary movements and sends signals to the striatum via nerve cells that release dopamine.其中一个主要投入纹状体来自地带，称为黑质，控制着随意运动，并将信号传递给经纹状体神经细胞释放多巴胺。

The researchers conducted various coordination tests on both normal and genetically manipulated mice.研究人员进行各种配合的测试都正常，并操纵基因小鼠。 Engineered mice given a drug that increased brain dopamine levels performed worse on these tasks, while TBZ protected against this effect.工程小鼠给予药物，增加脑内多巴胺水平的表现差于这些任务，而tbz保护，防止这种效应。 Most importantly, TBZ appears to reduce significantly cell loss in the striatum of the engineered mice, the scientists report.最重要的是， tbz出现大幅降低细胞损失在纹状体的老鼠，科学家们报告。

“More research is needed to determine whether this protective effect might also be present in humans, and also whether at-risk people would benefit from the drug,” Dr. Bezprozvanny said. "需要更多的研究来确定这是否保护作用可能也将出席在人类中，也无论是在高危人群中，将受惠于药物， "博士bezprozvanny说。

Clinical trials in humans would be very difficult, however, because trials require many participants and there is no easy way to score effectiveness of a presymptomatic drug, Dr. Bezprozvanny said.临床试验中，在人类将是非常困难的，但是，由于审判需要许多与会者并没有简单的方法来评分效力的presymptomatic药物，博士bezprozvanny说。 Thus, his future studies in animals will look at the effectiveness of tetrabenazine (TBZ) given just after initial symptoms of Huntington’s have developed.因此，其未来对动物的研究，将看成效嗪（ tbz ）由于刚刚经过初步的症状，亨廷顿的全面发展。