Article
Lymphoid differentiation of hematopoietic stem cells requires efficient Cxcr4 desensitization.
The Journal of experimental medicine - 3 Jul 2017
Freitas Christelle, Wittner Monika, Nguyen Julie, Rondeau Vincent, Biajoux Vincent, Aknin Marie-Laure, Gaudin Françoise, Beaussant-Cohen Sarah, Bertrand Yves, Bellanné-Chantelot Christine, Donadieu Jean, Bachelerie Françoise, Espéli Marion, Dalloul Ali, Louache Fawzia, Balabanian Karl
Abstract excerpt
The CXCL12/CXCR4 signaling exerts a dominant role in promoting hematopoietic stem and progenitor cell (HSPC) retention and quiescence in bone marrow. Gain-of-function CXCR4 mutations that affect homologous desensitization of the receptor have been reported in the WHIM Syndrome (WS), a rare immunodeficiency characterized by lymphopenia. The mechanisms underpinning this remain obscure. Using a mouse model with a...
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