| First Author | Glenthøj A | Year | 2014 |
| Journal | PLoS One | Volume | 9 |
| Issue | 3 | Pages | e92471 |
| PubMed ID | 24658030 | Mgi Jnum | J:214157 |
| Mgi Id | MGI:5588509 | Doi | 10.1371/journal.pone.0092471 |
| Citation | Glenthoj A, et al. (2014) Human alpha-defensin expression is not dependent on CCAAT/enhancer binding protein-epsilon in a murine model. PLoS One 9(3):e92471 |
| abstractText | Specific granule deficiency (SGD) is a rare congenital disorder characterized by recurrent infections. The disease is caused by inactivating mutations of the CCAAT/enhancer binding protein-epsilon (C/EBP-epsilon) gene. As a consequence, specific and gelatinase granules lack most matrix proteins. Furthermore, azurophil granules contain diminished amounts of their most abundant proteins, alpha-defensins, also known as human neutrophil peptides (HNPs). In accordance with this, in vitro models have demonstrated induction of HNPs by C/EBP-epsilon. Since mice do not express myeloid defensins, they cannot per se be used to characterize the role of C/EBP-epsilon in controlling HNP expression in vivo. We therefore crossed a transgenic HNP-1-expressing mouse with the Cebpe-/- mouse to study the in vivo significance of C/EBP-epsilon for HNP-1 transcription and expression. Surprisingly, neither expression nor processing of HNP-1 was affected by lack of C/EBP-epsilon in these mice. Transduction of C/EBP-epsilon into primary bone marrow cells from HNP-1 mice induced some HNP-1 expression, but not to levels comparable to expression human cells. Taken together, our data infer that the HNP-1 of the transgenic mouse does not show an expression pattern equivalent to endogenous secondary granule proteins. This limits the use of these transgenic mice as a model for human conditions. |
