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Hematologic disorder-associated Cxcr4 gain-of-function mutation leads to uncontrolled extrafollicular immune response.

Blood

June 2021

Ecotaxie, Microenvironnement et Developpement Lymphocytaire (EMiLy), INSERM U1160, Institut de Recherche Saint Louis, Université de Paris, Paris, France.

The extrafollicular immune response is essential to generate a rapid but transient wave of protective antibodies during infection. Despite its importance, the molecular mechanisms controlling this first response are poorly understood. Here, we demonstrate that enhanced Cxcr4 signaling caused by defective receptor desensitization leads to exacerbated extrafollicular B-cell response.

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