2024
DOI: 10.1073/pnas.2413162121
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Enforced CARD11/MALT1 signaling in dendritic cells triggers hemophagocytic lymphohistiocytosis

Sophie E. Isay,
Larsen Vornholz,
Theresa Schnalzger
et al.

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome fueled by uncontrolled mononuclear phagocyte activity, yet the innate immune mechanisms driving HLH pathogenesis remain elusive. Germline gain-of-function (GOF) mutations in CARD11, a pivotal regulator of lymphocyte antigen receptor signaling, cause the lymphoproliferative disease B-cell expansion with NF-κB and T-cell anergy, which is frequently associated with HLH development. Given that CARD11 is physiologically expressed not only in ly… Show more

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