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CHAI and LATAIE: new genetic diseases of CTLA-4 checkpoint insufficiency

2016; Elsevier BV; Volume: 128; Issue: 8 Linguagem: Inglês

10.1182/blood-2016-04-712612

1528-0020

Bernice Lo, Jill M. Fritz, Helen C. Su, Gülbû Uzel, Michael B. Jordan, Michael J. Lenardo,

Immune Cell Function and Interaction

Abstract 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.