Abstract
CARD11 functions as a key signaling scaffold that controls antigen-induced lymphocyte activation during the adaptive immune response. Somatic mutations in CARD11 are frequently found in Non-Hodgkin lymphoma, and at least three classes of germline CARD11 mutations have been described as the basis for primary immunodeficiency. In this review, we summarize our current understanding of how CARD11 signals, how its activity is regulated, and how mutations bypass normal regulation to cause disease.
Highlights
Our understanding of the immune system has benefited greatly from the study of the molecular circuitry in lymphocytes that governs antigen recognition and the cellular and systemic response to pathogens
Following activation, binding partners dissociate from CARD11 and the protein returns to the inactive state
CARD11 facilitates the polyubiquitinylation of IKKγ with K63linked chains in response to antigen receptor triggering [33], which is accomplished by a MALT1-associated E3 ligase activity [34]
Summary
Our understanding of the immune system has benefited greatly from the study of the molecular circuitry in lymphocytes that governs antigen recognition and the cellular and systemic response to pathogens. This circuitry is elaborate, highly regulated, sensitive, and specific. Receptor engagement leads to the conversion of CARD11 to an open, active scaffold that binds signaling partners. Following activation, binding partners dissociate from CARD11 and the protein returns to the inactive state This cycle, from closed to open and back to closed states, is thought to occur over the course of approximately 60 minutes following BCR or TCR engagement
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