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Blood. 2016 Aug 25;128(8):1037-42. doi: 10.1182/blood-2016-04-712612. Epub 2016 Jul 14.

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

Author information

1
Division of Translational Medicine, Sidra Medical and Research Center, Doha, Qatar; Molecular Development of the Immune System Section, Laboratory of Immunology, Clinical Genomics Program.
2
Molecular Development of the Immune System Section, Laboratory of Immunology, Clinical Genomics Program.
3
Clinical Genomics Program, Human Immunological Diseases Section, Laboratory of Host Defenses, and.
4
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD;
5
Division of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, and Division of Immunobiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH.

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.

PMID:
27418640
PMCID:
PMC5000841
DOI:
10.1182/blood-2016-04-712612
[Indexed for MEDLINE]
Free PMC Article

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