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@article{397101, author = {Wolf, HM. and Thon, Vojtěch and Lechleitner, S. and Eibl, Marta and Petzelbauer, P.}, article_number = {2}, keywords = {MHC class II deficiency; dendritic cells; endothelial cells; antigen presentation; residual cell type-specific MHC class II expression}, language = {eng}, issn = {0007-1048}, journal = {British Journal of Haematology}, title = {Residual expression of functional MHC class II molecules in twin brothers with MHC class II deficiency is cell type specific}, volume = {115}, year = {2001} }
TY - JOUR ID - 397101 AU - Wolf, HM. - Thon, Vojtěch - Lechleitner, S. - Eibl, Marta - Petzelbauer, P. PY - 2001 TI - Residual expression of functional MHC class II molecules in twin brothers with MHC class II deficiency is cell type specific JF - British Journal of Haematology VL - 115 IS - 2 SP - 460 EP - 460 SN - 00071048 KW - MHC class II deficiency KW - dendritic cells KW - endothelial cells KW - antigen presentation KW - residual cell type-specific MHC class II expression N2 - We examined major histocompatibility complex (MHC) class II expression in B cells, peripheral blood monocytes, activated T cells, epidermal Langerhans cells, monocyte-derived dendritic cells, dermal microvascular endothelial cells (DMEC) and fibroblasts of twin brothers with MHC class II deficiency. Although residual human leucocyte antigen (HLA)-DR expression was found on a subpopulation of epidermal Langerhans cells and a subset of peripheral blood monocyte-derived dendritic cells, the patients' B cells, monocytes and activated T cells were HLA-DR negative. After treatment with interferon-gamma (IFN-gamma), the patients' DMEC expressed HLA-DR but not -DP and -DQ at the protein and mRNA level, whereas IFN-gamma failed to induce HLA-DR expression on dermal fibroblasts. The patients' monocyte-derived dendritic cells were capable of processing and presenting tetanus toxoid to autologous T cells, and patient-derived DMEC induced the proliferation of allogeneic CD4(+) T cells in an MHC class II-restricted fashion, indicating that the observed residual MHC class II surface expression was functional. The findings reported show that the defect encountered in these patients is not necessarily expressed to the same extent in different cell lineages, which is relevant for the understanding of the patients' phenotype and also illustrates that only small amounts of MHC class II are needed to mount a functional cellular immune response in vivo. ER -
WOLF, HM., Vojtěch THON, S. LECHLEITNER, Marta EIBL a P. PETZELBAUER. Residual expression of functional MHC class II molecules in twin brothers with MHC class II deficiency is cell type specific. \textit{British Journal of Haematology}. 2001, roč.~115, č.~2, s.~460-471. ISSN~0007-1048.
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