对蛋白质杀人案:好的,坏的和丑陋的

白细胞释放抗体,淋巴细胞对抗传染病

The boy’s disease became known as X-linked a-gammaglobulin anemia – or Bruton’s Agammaglobulinemia – and its identification was heralded as an important medical discovery, even being featured as such by TIME magazine in 1953.

It would take another 40 years before the enzyme responsible for the syndrome was identified and aptly named Bruton’s tyrosine kinase, or BTK.

“From Dr. Bruton’s discovery, we could understand the role of BTK because of what the human deficiency does to antibody production – without BTK, we don’t produce antibodies,” says Andy Long, Ph.D., senior principal research scientist at the AbbVie Bioresearch Center in Worcester, Massachusetts, U.S.A.

奥格登布鲁顿博士是一名儿科医生在沃尔特里德陆军医院在1950年代早期,当他第一次看到一个小男孩出现复发性肺部感染。测试显示,男孩没有循环血液中的抗体,使他的身体很难对抗感染。

男孩的病被称为x连锁a-gammaglobulin贫血-或布鲁顿Agammaglobulinemia及其识别被称为是一个重要的医学发现,甚至出现这样的时间杂志在1953年。

需要另一个40年前酶负责综合症是识别和名为布鲁顿的酪氨酸激酶,或对。杀人案

“从布鲁顿博士的发现,我们能理解的角色对因为杀人案抗体生产——没有什么人类缺乏对,杀人案我们不产生抗体,”安迪说,博士,高级首席研究科学家AbbVie伍斯特生物学研究中心,美国马萨诸塞州

The good: BTK has the critical role of activating B cells leading to maturation and growth. The mature B cells can then produce needed antibodies. In a normal setting, this is a self-limiting process.

There are times, however, when the process goes awry.

The bad: Sometimes BTK is abnormally and persistently activated, signaling the B cells to grow and proliferate uncontrollably because there is no shut-off switch.

The ugly: The excess B cells crowd out the normal cells in the bone marrow and lymph nodes, and leukemia or lymphoma – cancers in the blood cells that are part of the immune system – can develop.

“In certain cancers, we have found that blocking BTK is one way to control the overproduction of B cells,” says Danelle James, M.D., M.S., head of clinical science at Pharmacyclics, an AbbVie company. “Our research has shown that targeting the BTK protein may be beneficial in certain B cell cancers, and we are hopeful that ongoing research will show that this mechanism also can help even more patients.”

也被称为B淋巴细胞,B细胞是白细胞的主要作用是产生抗体,帮助身体抵抗感染。

良好的:对有杀人案激活B细胞的关键作用导致成熟和成长。所需的成熟B细胞可以产生抗体。在正常的环境中,这是一个自我的过程。

有时,然而,当这个过程出错。

坏:有时对异常持续激活,杀人案信号B细胞生长并增殖失控,因为没有关闭开关。

丑陋的:多余的B细胞排挤正常细胞在骨髓和淋巴结,癌症和白血病或淋巴瘤——血液中的细胞是免疫系统的一部分,可以开发。

“在某些癌症,我们发现,阻断对是一种控制杀人案B细胞的生产过剩,“Danelle詹姆斯说,医学博士,硕士AbbVie Pharmacyclics临床科学主管,公司。“我们的研究表明,针对对蛋白质杀人案在某些B细胞癌症可能是有益的,和我们希望正在进行的研究表明,这种机制也可以帮助更多的病人。”

The good: In a normal setting, after getting the signal from BTK, mature B cells would activate and produce antibodies to fight off a particular infection.

The bad: In autoimmunity, B cells start to produce antibodies against normal proteins in the body (known as auto-antibodies.) In other words, BTK activation is telling the bad B cells to generate antibodies to the wrong thing. BTK is also involved in how the body reacts to those autoantibodies.

The ugly: In autoimmunity, when BTK causes the dysregulated production of auto-antibodies it can result in destruction of normal tissue rather than the infection, leading to diseases such as rheumatoid arthritis (RA).

“When we look at what this means for patients with autoimmune diseases, we are trying to block the activated BTK in order to shut down the production of those auto-antibodies and stop the reaction to those antibodies in the tissue,” Dr. Long says. “We know multiple pathways are involved in autoimmune diseases like RA and we are researching new medicines that combine different mechanisms so that we might be able to bring forward treatments that ultimately may benefit more patients.”

在自身免疫,正常流程涉及对也不安,杀人案而是B细胞的过度增殖,使身体的信号本身。

良好的:在正常的环境中,从对,杀人案得到信号后成熟的B细胞会激活并产生抗体对抗感染。

坏B:在自身免疫,细胞开始产生抗体正常蛋白质在体内(称为auto-antibodies。)换句话说,对激活杀人案告诉坏的B细胞产生抗体来错了。对还参与杀人案身体如何对这些自身抗体。

丑陋的:在自身免疫,对导致生产auto-antibodies特异表达杀人案时可能导致破坏正常组织而不是感染,导致疾病,如类风湿性关节炎(RA)。

“当我们看看这意味着患者的自身免疫性疾病,我们正在努力阻止激活对为了杀人案关闭生产那些auto-antibodies和停止反应抗体的组织,“长博士说。“我们知道多个通路参与自身免疫性疾病如风湿性关节炎和我们正在研究新的药物,结合不同的机制,这样我们可以提前治疗,最终可能受益更多的病人。”

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