The human syndrome of dendritic cell, monocyte, B and NK lymphoid deficiency
| العنوان: | The human syndrome of dendritic cell, monocyte, B and NK lymphoid deficiency |
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| المؤلفون: | Matthew Collin, Rachel E. Dickinson, Jonathan P. Wallis, Muzlifah Haniffa, Xiao-Nong Wang, Sarah Pagan, Naomi McGovern, Laura Jardine, Andrew J. Cant, Cliff Morgan, Venetia Bigley, Mirjam van der Burg, Sergei Doulatov, Jacques J.M. van Dongen, Sophie Hambleton, John E. Dick, James L. Lordan, Ignatius Chua, Rainer Doffinger, Dinakantha S. Kumararatne, Ian Dimmick |
| المساهمون: | Immunology |
| المصدر: | The Journal of Experimental Medicine Journal of Experimental Medicine, 208(2), 227-234. Rockefeller University Press |
| سنة النشر: | 2011 |
| مصطلحات موضوعية: | Adult, CD4 antigen, CD14, Immunology, CD11c, chemical and pharmacologic phenomena, Bone Marrow Cells, Enzyme-Linked Immunosorbent Assay, Biology, Monocytes, Interferon-gamma, chemistry.chemical_compound, Interleukin 21, Antigens, CD, hemic and lymphatic diseases, medicine, Humans, Immunology and Allergy, Child, Mycobacterium Infections, Follicular dendritic cells, Monocyte, Brief Definitive Report, hemic and immune systems, Dendritic Cells, HLA-DR Antigens, Leukopenia, Syndrome, Dendritic cell, Flow Cytometry, Molecular biology, medicine.anatomical_structure, Microscopy, Fluorescence, chemistry, Disease Susceptibility, Bone marrow |
| الوصف: | Human immunodeficiency syndrome with loss of DCs, monocytes, and T reg cells; preservation of Langerhans cells; associated loss of BM multilymphoid progenitors; and overproduction of Flt3 ligand. Congenital or acquired cellular deficiencies in humans have the potential to reveal much about normal hematopoiesis and immune function. We show that a recently described syndrome of monocytopenia, B and NK lymphoid deficiency additionally includes the near absence of dendritic cells. Four subjects showed severe depletion of the peripheral blood HLA-DR+ lineage− compartment, with virtually no CD123+ or CD11c+ dendritic cells (DCs) and very few CD14+ or CD16+ monocytes. The only remaining HLA-DR+ lineage− cells were circulating CD34+ progenitor cells. Dermal CD14+ and CD1a+ DC were also absent, consistent with their dependence on blood-derived precursors. In contrast, epidermal Langerhans cells and tissue macrophages were largely preserved. Combined loss of peripheral DCs, monocytes, and B and NK lymphocytes was mirrored in the bone marrow by complete absence of multilymphoid progenitors and depletion of granulocyte-macrophage progenitors. Depletion of the HLA-DR+ peripheral blood compartment was associated with elevated serum fms-like tyrosine kinase ligand and reduced circulating CD4+CD25hiFoxP3+ T cells, supporting a role for DC in T reg cell homeostasis. |
| تدمد: | 0022-1007 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::449b7e88c0dedf8077566182309904a3 https://doi.org/10.1084/jem.20101459 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....449b7e88c0dedf8077566182309904a3 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 00221007 |
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