One of the causes of inborn errors of immunity is immune dysregulation. The inability of the immune system to regulate the extent of its activity has several deleterious effects, including autoimmunity, recurrent infections, and malignancy. In recent years, many proteins in the CD28 family -CD28, ICOS, CTLA-4, PD-1, and BTLA -have come into the focus of several research areas for their consequential role in the upregulation or downregulation of the immune response. In this review, we will discuss the structure and function of these proteins, as well as provide an overview of the clinical picture of patients with genetic defects.
Die CD28-Rezeptorfamilie spielt eine zentrale Rolle bei der Modulation der Immunantwort. Durch ein grundlegendes Verständnis der beteiligten Signalwege und Partner konnten Kliniker nicht nur die Therapieoptionen für eine Reihe von Autoimmunerkrankungen und Immundefizienzen erweitern, sondern auch den Grundstein für eine ganz neue Kategorie von Krebsbehandlungen legen. In dieser Übersichtsarbeit werden wir einige grundlegende Informationen zu den Signalwegen, den murinen Modellen und der klinischen Relevanz der einzelnen Rezeptoren der CD28-Familie geben.
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