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Discoordinate Expression of Invariant Chain and MHC Class II Genes in Class II Transactivator-Transfected Fibroblasts Defective for RFX5¹

Ad Peijnenburg^*, Marja J. C. A. Van Eggermond^*, Sam J. P. Gobin^*, Rian Van den Berg^*, Barbara C. Godthelp^*, Jaak M. J. J. Vossen and Peter J. Van den Elsen^2,*

Departments of ^* Immunohematology and Blood Bank and Pediatrics, Leiden University Medical Center, Leiden, The Netherlands

MHC class II deficiency or bare lymphocyte syndrome is a severe combined immunodeficiency caused by defects in MHC-specific transcription factors. In the present study, we show that fibroblasts derived from a recently identified bare lymphocyte syndrome patient, SSI, were mutated for RFX5, one of the DNA-binding components of the RFX complex. Despite the lack of functional RFX5 and resulting MHC class II-deficient phenotype, transfection of exogenous class II transactivator (CIITA) in these fibroblasts can overcome this defect, resulting in the expression of HLA-DR, but not of DP, DQ, and invariant chain. The lack of invariant chain expression correlated with lack of CIITA-mediated transactivation of the invariant chain promoter in transient transfection assays in SSI fibroblast cells. Consequently, these CIITA transfectants lacked Ag-presenting functions.

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