Dysregulated Stem Cell Niches and Altered Lymphocyte Recirculation Cause B and T Cell Lymphopenia in WHIM Syndrome

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Using a CXCR4 R334X gain-of-function mouse model of WHIM syndrome, scientists showed that lymphopoiesis is reduced because of a dysregulated mesenchymal stem cell transcriptome characterized by a switch from an adipogenic to an osteolineage-prone program with limited lymphopoietic activity.
[Science Immunology]
Abstract