The role of TACI in the molecular pathogenesis of Common Variable immunodeficiency

TACI 在常见变异型免疫缺陷分子发病机制中的作用

• 批准号: G0501468/1
• 负责人: Hubert Gaspar
• 金额: $ 94.76万
• 依托单位: University College London
• 依托单位国家: 英国
• 项目类别: Research Grant
• 财政年份: 2006
• 资助国家: 英国
• 起止时间: 2006 至 无数据
• 项目状态: 已结题
• 来源: https://gtr.ukri.org/projects?ref=G0501468%2F1
• 关键词: role; TACI; molecular; pathogenesis; Common

Common variable immunodeficiency (CVID) is a condition which affects the immune system and results in an inability to fight infection, problems with autoimmunity and in some cases development of lymphoma. The gene defect in CVID has only recently been identified. The gene, TACI, is abnormal in CVID but we do not as yet understand why TACI defects leads to the development of the disease. We will first check over 400 CVID patients for abnormalities in TACI so that we can understand how frequent these abnormalities are. Next we plan to look at how abnormal TACI protein works and functions when placed in combination with normal TACI proteins. We also plan to create a mouse model where the TACI gene has been made defective in a way similar to the human condition. This will provide us with a living system where we can examine the effects of TACI defects on the immune system. A greater understanding of these TACI defects will allow us to develop ways of treating CVID in the future.

共同变量免疫缺陷(CVID)是一种影响免疫系统的疾病，导致无法抵抗感染、自身免疫问题，在某些情况下还会发展成淋巴瘤。CVID中的基因缺陷直到最近才被发现。TACI基因在CVID中是异常的，但我们还不清楚TACI缺陷导致疾病发展的原因。我们将首先检查400多名CVID患者的TACI异常，以便了解这些异常的频率有多高。接下来，我们计划观察异常的TACI蛋白与正常的TACI蛋白结合时是如何工作和功能的。我们还计划创建一种小鼠模型，其中TACI基因已经以一种类似于人类情况的方式发生了缺陷。这将为我们提供一个活的系统，在那里我们可以检查TACI缺陷对免疫系统的影响。对这些TACI缺陷的更好理解将使我们能够在未来开发治疗CVID的方法。