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

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...

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Veröffentlicht in:	Blood 2016-08, Vol.128 (8), p.1037-1042
Hauptverfasser:	Lo, Bernice, Fritz, Jill M., Su, Helen C., Uzel, Gulbu, Jordan, Michael B., Lenardo, Michael J.
Format:	Artikel
Sprache:	eng
Schlagworte:	Cell Cycle Checkpoints - genetics CTLA-4 Antigen - genetics Humans Immune System Diseases - genetics Immune System Diseases - therapy Immunotherapy Mutation - genetics Phenotype Spotlight
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Beschreibung
Zusammenfassung:	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.
ISSN:	0006-4971 1528-0020
DOI:	10.1182/blood-2016-04-712612