美国国家公共电台 NPR--Encore: Scientists look to people with Down syndrome to test Alzheimer's drugs

Encore: Scientists look to people with Down syndrome¹ to test Alzheimer's drugs

Transcript²

People with Down syndrome are sought after for Alzheimer's research studies because many develop it in their 40s and 50s. Most will get it if they live long enough. (Aired on ATC on July 7, 2022.)

LEILA FADEL, HOST:

Alzheimer's disease poses a major threat to people with Down syndrome. Many develop the disease in their 40s and 50s, and most will get it if they live long enough. People with Down syndrome may help researchers find treatments for Alzheimer's, as NPR's Jon Hamilton reports.

JON HAMILTON, BYLINE³: Frank Stephens is an actor, a writer and an advocate for people with Down syndrome.

FRANK STEPHENS: I am a man with a very good life. I have a beautiful girlfriend.

HAMILTON: Stephens, who is 40, also has a major concern. His mother is in the late stages of Alzheimer's.

STEPHENS: She is now - almost childlike now. It's very hard to see.

HAMILTON: And as a person with Down syndrome, Stephens is aware that he is likely to develop Alzheimer's himself. So he raises money for Alzheimer's research through the Global Down Syndrome Foundation, and he's part of a research effort called the Human Trisome Project.

STEPHENS: I did take a blood test. I took it to see whether or not I may end up with Alzheimer's.

HAMILTON: Stephens' goal is to help scientists find a drug that can defeat Alzheimer's.

STEPHENS: That would be amazing. And I hope that not only people with Down syndrome can take it, but also people without Down syndrome can take it, too. And I'm hoping I can do that for my mother.

HAMILTON: People with Down syndrome carry an extra copy of chromosome⁴ 21. That causes intellectual disability. It also changes the brain in at least two ways that can lead to Alzheimer's.

JOAQUIN ESPINOSA: People with Down syndrome give us a unique opportunity to understand what modulates⁵ the severity and the progress of Alzheimer's disease.

HAMILTON: Joaquin Espinosa directs the Linda Crnic Institute for Down Syndrome in Aurora⁶, Colo. He says people with the condition have a hyperactive immune system that protects them from some cancers but also leads to chronic⁷ inflammation.

ESPINOSA: And of importance to Alzheimer's, they have brain inflammation across the lifespan.

HAMILTON: There's growing evidence that brain inflammation plays an important role in Alzheimer's. So Espinosa and a team of researchers are looking for ways to keep the brain's immune system in check.

ESPINOSA: We are running clinical trials for immune modulating⁸ agents in Down syndrome. So there is an active trial right now to turn down that response with a class of drugs known as JAK inhibitors.

HAMILTON: That's J-A-K. JAK inhibitors are used to reduce inflammation in the joints⁹ of people with rheumatoid arthritis¹⁰. Espinosa hopes these drugs can do the same thing in the brain and cut the risk of Alzheimer's. So he's trying the approach in people with Down syndrome.

Another team at the Crnic Institute is taking a different approach to modulating the immune system. Dr. Huntington Potter says the idea is to boost a special immune cell found in the brain.

HUNTINGTON POTTER: And its job is to eat up things that aren't supposed to be there, like amyloid.

HAMILTON: Amyloid is the sticky substance that builds up in the brains of people with Alzheimer's. And that's the second reason people with Down syndrome are vulnerable to the disease. The extra chromosome they carry causes the brain to produce extra amyloid. Potter hopes to counter this with a drug called leukine, which increases the immune cells that eat amyloid. Last year, he did a small study to establish that leukine could safely be given to people with Alzheimer's.

POTTER: We did not expect to see a cognitive¹¹ benefit. But three weeks of treatment of leukine, and the individuals actually improved in their cognition.

HAMILTON: Those people didn't have Down syndrome. But Potter says in March, his team showed that leukine also worked in mice that did have Down syndrome.

POTTER: That then allowed us to apply for a grant to study leukine in young adults with Down syndrome before they get Alzheimer's disease.

HAMILTON: They got the grant. Now they're preparing to recruit young adults with Down syndrome. Lina Patel, a psychologist at the Crnic Institute, is confident that people will enroll¹² in the study.

LINA PATEL: The self-advocates that we work with really are proponents¹³ because I think that they do see that it is directly impacting their lives.

HAMILTON: And the lives of others. The scientists expect to know whether leukine works in the next five years or so.

Jon Hamilton, NPR News.

1 syndrome   

n.综合病症；并存特性

参考例句：

• The Institute says that an unidentified virus is to blame for the syndrome. 该研究所表示,引起这种综合症的是一种尚未确认的病毒。
• Results indicated that 11 fetuses had Down syndrome. 结果表明有11个胎儿患有唐氏综合征。

2 transcript   

n.抄本，誊本，副本，肄业证书

参考例句：

• A transcript of the tapes was presented as evidence in court.一份录音带的文字本作为证据被呈交法庭。
• They wouldn't let me have a transcript of the interview.他们拒绝给我一份采访的文字整理稿。

3 byline   

n.署名；v.署名

参考例句：

• His byline was absent as well.他的署名也不见了。
• We wish to thank the author of this article which carries no byline.我们要感谢这篇文章的那位没有署名的作者。

4 chromosome   

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。

5 modulates   

调整( modulate的第三人称单数 )； （对波幅、频率的）调制； 转调； 调整或改变（嗓音）的音调

参考例句：

• At this point the music modulates from C to G. 在此处音乐从C调转到G调。
• Modulates the level of adrenaline to relieve stress and fatigue. 剌激肾上腺素的分泌，有助纾缓压力、消除疲劳。

6 aurora   

n.极光

参考例句：

• The aurora is one of nature's most awesome spectacles.极光是自然界最可畏的奇观之一。
• Over the polar regions we should see aurora.在极地高空,我们会看到极光。

7 chronic   

adj.(疾病)长期未愈的，慢性的；极坏的

参考例句：

• Famine differs from chronic malnutrition.饥荒不同于慢性营养不良。
• Chronic poisoning may lead to death from inanition.慢性中毒也可能由虚弱导致死亡。

8 modulating   

调整( modulate的现在分词 )； （对波幅、频率的）调制； 转调； 调整或改变（嗓音）的音调

参考例句：

• In his horn solo,he kept modulating from key to key. 他在喇叭独奏时不断地变调。
• Water vapour and clouds are the primary variables modulating direct solar absorption. 水汽和云是调节直接太阳吸收的主要要素。

9 joints   

接头( joint的名词复数 )； 关节； 公共场所（尤指价格低廉的饮食和娱乐场所） （非正式）； 一块烤肉 （英式英语）

参考例句：

• Expansion joints of various kinds are fitted on gas mains. 各种各样的伸缩接头被安装在煤气的总管道上了。
• Expansion joints of various kinds are fitted on steam pipes. 各种各样的伸缩接头被安装在蒸气管道上了。

10 arthritis   

n.关节炎

参考例句：

• Rheumatoid arthritis has also been linked with the virus.风湿性关节炎也与这种病毒有关。
• He spent three months in the hospital with acute rheumatic arthritis.他患急性风湿性关节炎，在医院住了三个月。

11 cognitive   

adj.认知的，认识的，有感知的

参考例句：

• As children grow older,their cognitive processes become sharper.孩子们越长越大，他们的认知过程变得更为敏锐。
• The cognitive psychologist is like the tinker who wants to know how a clock works.认知心理学者倒很像一个需要通晓钟表如何运转的钟表修理匠。

12 enroll   

v.招收；登记；入学；参军；成为会员（英)enrol

参考例句：

• I should like to enroll all my children in the swimming class.我愿意让我的孩子们都参加游泳班。
• They enroll him as a member of the club.他们吸收他为俱乐部会员。

13 proponents   

n.（某事业、理论等的）支持者，拥护者( proponent的名词复数 )

参考例句：

• Reviewing courts were among the most active proponents of hybrid rulemaking procedures. 复审法院是最积极的混合型规则制定程序的建议者。 来自英汉非文学 - 行政法
• Proponents of such opinions were arrested as 'traitors. ' 提倡这种主张的人马上作为“卖国贼”逮捕起来。 来自辞典例句