期刊
CELL
卷 183, 期 7, 页码 1826-+出版社
CELL PRESS
DOI: 10.1016/j.cell.2020.10.046
关键词
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资金
- National Center for Advancing Translational Sciences of the National Institutes of Health [UL1TR001866]
- National Institute of Allergy and Infectious Diseases [R37AI095983, U19AI118626]
- Sackler Center for Biomedicine and Nutrition at the Center for Clinical and Translational Science (CCTS), Shapiro-Silverberg Fund for the Advancement of Translational Research at CCTS, Rockefeller University
- Integrative Biology of Emerging Infectious Diseases Laboratory of Excellence [ANR-10-LABX-62-IBEID]
- French National Research Agency under the Investments for the future'' program [ANR-10-IAHU-01]
- ANR-GENMSMD [ANR-16-CE17-0005-01]
- ANR-LTh-MSMD-CMCD [ANR-18-CE93-0008-01]
- French Foundation for Medical Research (FRM) [EQU201903007798]
- SCOR Corporate Foundation for Science, ECOS Nord [C19S01-63407]
- Swiss National Science Foundation [310030L_182728]
- INSERM Poste d'accueil
- Imagine Institute
- Qatar National Research Fund [PPM1-1220150017]
- National Health and Medical Research Council of Australia [1113904, 1042925, 1176665]
- Early/MidCareer Development Research Fellowship, Ministry of Health, NSW Government
- Stony Wold-Herbert Fund
- National Health and Medical Research Council of Australia [1176665] Funding Source: NHMRC
- Swiss National Science Foundation (SNF) [310030L_182728] Funding Source: Swiss National Science Foundation (SNF)
Inborn errors of human interferon gamma (IFN-gamma) immunity underlie mycobacterial disease. We report a patient with mycobacterial disease due to inherited deficiency of the transcription factor T-bet. The patient has extremely low counts of circulating Mycobacterium-reactive natural killer (NK), invariant NKT (iNKT), mucosal-associated invariant T (MATT), and V delta 2(+) gamma delta T lymphocytes, and of Mycobacterium-non reactive classic T(H)1 lymphocytes, with the residual populations of these cells also producing abnormally small amounts of IFN-gamma. Other lymphocyte subsets develop normally but produce low levels of IFN-gamma, with the exception of CD8(+) alpha beta T and non-classic CD4(+) cep T(H)1* lymphocytes, which produce IFN-gamma normally in response to mycobacterial antigens. Human T-bet deficiency thus underlies mycobacterial disease by preventing the development of innate (NK) and innate-like adaptive lymphocytes (iNKT, MATT, and V delta 2(+) gamma delta T cells) and IFN-gamma production by them, with mycobacterium-specific, IFN-gamma-producing, purely adaptive CD8(+) alpha beta T and CD4(+) alpha beta T(H)1* cells unable to compensate for this deficit.
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