Granulocyte-Macrophage colony
stimulating factor can salvage JAGN1 defect in bone marrow precursors causing
congenital neutropenia. Neutrophils are the sentinel of host immune systems
that are dispatched to surveil, engage and combat microbial pathogens.
Neutropenic
patients with low neutrophil counts are unable to mount neutrophil based
immune responses and as a result become vulnerable to infections. JAGN1
encoding Jagunal homolog 1 expressed in hematopoietic progenitors and it’s
deficiency makes neutrophil incompetent effector cells.
In JAGN1 mutant mice, Penniger et
al. demonstrated that the defect in JAGN1 function is rescued by granulocyte/
macrophage colony stimulating factor (GM-CSF) when afflicted by Candida
albicans.
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