| First Author | Clausen BE | Year | 1998 |
| Journal | Immunity | Volume | 8 |
| Issue | 2 | Pages | 143-55 |
| PubMed ID | 9491996 | Mgi Jnum | J:67941 |
| Mgi Id | MGI:1931718 | Doi | 10.1016/s1074-7613(00)80467-7 |
| Citation | Clausen BE, et al. (1998) Residual MHC class II expression on mature dendritic cells and activated B cells in RFX5-deficient mice. Immunity 8(2):143-55 |
| abstractText | Patients with major histocompatibility complex class II (MHC-II) deficiency are known to carry mutations in either the RFX complex or the trans-activator CIITA. While the pivotal role of CIITA for MHC-II gene transcription is supported by the essential absence of MHC-II molecules in CIITA-deficient mice, we demonstrate here that RFX5-/- mice retain expression of MHC-II in thymic medulla, mature dendritic cells, and activated B cells. Nevertheless, RFX5-/- mice develop a severe immunodeficiency due to the lack of MHC-II in thymic cortex, failure of positive selection of CD4+ T cells, and absence of MHC-II on resting B cells and resident or IFNgamma-activated macrophages. This differential requirement for CIITA and RFX5 in subsets of antigen-presenting cells may be specific for the mouse; it may, however, also exist in humans without having been noticed so far. |
