WHIM syndrome myelokathexis reproduced in the NOD/SCID mouse xenotransplant model engrafted with healthy human stem cells transduced with C-terminus-truncated CXCR4.

@article{Kawai2007WHIMSM,
  title={WHIM syndrome myelokathexis reproduced in the NOD/SCID mouse xenotransplant model engrafted with healthy human stem cells transduced with C-terminus-truncated CXCR4.},
  author={Toshinao Kawai and Uimook Choi and Lanise Cardwell and Suk See DeRavin and Nora Naumann and Narda L. Whiting-Theobald and Gilda F. Linton and Jaehyun Moon and Philip G. M. Murphy and Harry L Malech},
  journal={Blood},
  year={2007},
  volume={109 1},
  pages={78-84}
}
WHIM(warts, hypogammaglobulinemia, recurrent bacterial infection, and myelokathexis) syndrome is a rare immunodeficiency caused in many cases by autosomal dominant C-terminal truncation mutations in the chemokine receptor CXCR4. A prominent and unexplained feature of WHIM is myelokathexis (hypercellularity with apoptosis of mature myeloid cells in bone marrow and neutropenia). We transduced healthy human CD34(+) peripheral blood-mobilized stem cells (PBSCs) with retrovirus vector encoding wild… CONTINUE READING

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