Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0348321 (
Haemophilus
)
15,372
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Eosinophil peroxidase
(
EPX
) is a major constituent of the large cytoplasmic granules of both human and mouse eosinophilic leukocytes. Human
EPX
deficiency is a rare, autosomal-recessive disorder limited to the eosinophil lineage. Our intent was to explore the impact of
EPX
gene deletion on eosinophil content, structure, and function. In response to repetitive intranasal challenge with a filtrate of the allergen, Alternaria alternata, we found significantly fewer eosinophils peripherally and in the respiratory tracts of
EPX
-/-
mice compared to wild-type controls; furthermore, both the major population (Gr1
-/lo
) and the smaller population of Gr1
hi
eosinophils from
EPX
-/-
mice displayed lower median fluorescence intensities (MFIs) for Siglec F. Quantitative evaluation of transmission electron micrographs of lung eosinophils confirmed the relative reduction in granule outer matrix volume in cells from the
EPX
-/-
mice, a finding analogous to that observed in human
EPX
deficiency. Despite the reduced size of the granule matrix, the cytokine content of eosinophils isolated from allergen-challenged
EPX
-/
-
and wild-type mice were largely comparable to one another, although the
EPX
-/-
eosinophils contained reduced concentrations of IL-3. Other distinguishing features of lung eosinophils from allergen-challenged
EPX
-/-
mice included a reduced fraction of surface TLR4
+
cells and reduced MFI for NOD1. Interestingly, the
EPX
gene deletion had no impact on eosinophil-mediated clearance of gram-negative
Haemophilus
influenzae from the airways. As such, although no clinical findings have been associated with human
EPX
deficiency, our findings suggest that further evaluation for alterations in eosinophil structure and function may be warranted.
...
PMID:Impact of eosinophil-peroxidase (EPX) deficiency on eosinophil structure and function in mouse airways. 3028 91
