Article
CXCR4-Specific Nanobodies as Potential Therapeutics for WHIM syndrome.
The Journal of pharmacology and experimental therapeutics - 1 Oct 2017
de Wit Raymond H, Heukers Raimond, Brink Hendrik J, Arsova Angela, Maussang David, Cutolo Pasquale, Strubbe Beatrijs, Vischer Henry F, Bachelerie Françoise, Smit Martine J
Abstract excerpt
WHIM syndrome is a rare congenital immunodeficiency disease, named after its main clinical manifestations: warts, hypogammaglobulinemia, infections, and myelokathexis, which refers to abnormal accumulation of mature neutrophils in the bone marrow. The disease is primarily caused by C-terminal truncation mutations of the chemokine receptor CXCR4, giving these CXCR4-WHIM mutants a gain of function in response to...
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