Lo Bernice, Fritz Jill M, Su Helen C, Uzel Gulbu, Jordan Michael B, Lenardo Michael J
Division of Translational Medicine, Sidra Medical and Research Center, Doha, Qatar; Molecular Development of the Immune System Section, Laboratory of Immunology, Clinical Genomics Program.
Molecular Development of the Immune System Section, Laboratory of Immunology, Clinical Genomics Program.
Blood. 2016 Aug 25;128(8):1037-42. doi: 10.1182/blood-2016-04-712612. Epub 2016 Jul 14.
CTLA-4 is a critical inhibitory "checkpoint" molecule of immune activation. Several recent reports have described patients with immune dysregulation and lymphoproliferative disease resulting from 2 different genetic diseases that directly or indirectly cause CTLA-4 deficiency. Numerous articles have also been published describing CTLA-4 blockade in cancer immunotherapy and its side effects, which are ultimately the consequence of treatment-induced CTLA-4 deficiency. Here, we review these 2 diseases and CTLA-4 blockade therapy, emphasizing the crucial role of CTLA-4 in immune checkpoint regulation.
CTLA-4是免疫激活的关键抑制性“检查点”分子。最近的几份报告描述了因两种直接或间接导致CTLA-4缺乏的不同遗传疾病而出现免疫失调和淋巴增殖性疾病的患者。也发表了许多文章描述癌症免疫治疗中CTLA-4阻断及其副作用，这些副作用最终是治疗诱导的CTLA-4缺乏的结果。在此，我们综述这两种疾病和CTLA-4阻断疗法，强调CTLA-4在免疫检查点调节中的关键作用。
