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- W4207030291 abstract "T lymphocytes are central cells of adaptive immunity. Activation of T lymphocytes by the antigen receptor of T cells (TCR) and co-stimulatory molecules involve specific signaling components and cascades. Those are essential for development, differentiation and effector responses of T lymphocytes. Over the last three decades, identification of primary immunodeficiencies associated with defects in development and activation of T lymphocytes provided new and unexpected insights into TCR signaling and co-signalling and their relation with protective immunity in humans. Mutations in components of the proximal and distal TCR signaling like the TCR-CD3 complex, protein tyrosine kinases and phosphatases, adaptor proteins, second messengers like Ca2+ mobilization and the MAPK kinase and nuclear factor kappa B (NFκB) pathways impede T cell development and functions, causing immunodeficiency and immune dysregulation manifestations such as autoimmunity and inflammation. Mutations that impair co-signaling delivers by co-stimulatory molecules of the tumor necrosis factor (TNF), the CD28 and the signaling lymphocytic activation molecule (SLAM) receptor families, have no effect or slight impact on T-cell development but impair T cell responses such as expansion. Interestingly, these latter are often associated with infectious susceptibility restricted to particular pathogens like Epstein-Barr virus (EBV) and human papillomavirus (HPV), highlighting the molecular specialization of co-stimulatory molecules to shape TCR-dependent T cell responses to specific pathogens or infected cells." @default.
- W4207030291 created "2022-01-26" @default.
- W4207030291 creator A5080495909 @default.
- W4207030291 date "2022-04-01" @default.
- W4207030291 modified "2023-10-13" @default.
- W4207030291 title "Inherited immunodeficiencies associated with proximal and distal defects in T cell receptor signaling and co-signaling" @default.
- W4207030291 cites W1482065222 @default.
- W4207030291 cites W1554199623 @default.
- W4207030291 cites W1581650643 @default.
- W4207030291 cites W1594461151 @default.
- W4207030291 cites W1644889756 @default.
- W4207030291 cites W1726495715 @default.
- W4207030291 cites W1964550750 @default.
- W4207030291 cites W1968247913 @default.
- W4207030291 cites W1976111921 @default.
- W4207030291 cites W1976594342 @default.
- W4207030291 cites W1980422861 @default.
- W4207030291 cites W1985508003 @default.
- W4207030291 cites W1993781709 @default.
- W4207030291 cites W2000433774 @default.
- W4207030291 cites W2001705316 @default.
- W4207030291 cites W2005487115 @default.
- W4207030291 cites W2019670504 @default.
- W4207030291 cites W2019737732 @default.
- W4207030291 cites W2025683558 @default.
- W4207030291 cites W2035754589 @default.
- W4207030291 cites W2046185478 @default.
- W4207030291 cites W2047301656 @default.
- W4207030291 cites W2047465993 @default.
- W4207030291 cites W2049553378 @default.
- W4207030291 cites W2052090039 @default.
- W4207030291 cites W2055221600 @default.
- W4207030291 cites W2055444005 @default.
- W4207030291 cites W2063645026 @default.
- W4207030291 cites W2066763901 @default.
- W4207030291 cites W2068208382 @default.
- W4207030291 cites W2074342072 @default.
- W4207030291 cites W2077322206 @default.
- W4207030291 cites W2086116383 @default.
- W4207030291 cites W2090203797 @default.
- W4207030291 cites W2090210678 @default.
- W4207030291 cites W2091954081 @default.
- W4207030291 cites W2094045649 @default.
- W4207030291 cites W2096879752 @default.
- W4207030291 cites W2098465830 @default.
- W4207030291 cites W2100412095 @default.
- W4207030291 cites W2105087340 @default.
- W4207030291 cites W2108671597 @default.
- W4207030291 cites W2114470082 @default.
- W4207030291 cites W2122028701 @default.
- W4207030291 cites W2124555673 @default.
- W4207030291 cites W2125337252 @default.
- W4207030291 cites W2125404510 @default.
- W4207030291 cites W2130473166 @default.
- W4207030291 cites W2132943662 @default.
- W4207030291 cites W2135163046 @default.
- W4207030291 cites W2137424665 @default.
- W4207030291 cites W2145687779 @default.
- W4207030291 cites W2154724678 @default.
- W4207030291 cites W2155864206 @default.
- W4207030291 cites W2163789582 @default.
- W4207030291 cites W2163956045 @default.
- W4207030291 cites W2167895056 @default.
- W4207030291 cites W2168448733 @default.
- W4207030291 cites W2168886601 @default.
- W4207030291 cites W2340157552 @default.
- W4207030291 cites W2396023876 @default.
- W4207030291 cites W2412861620 @default.
- W4207030291 cites W2466190463 @default.
- W4207030291 cites W2512972788 @default.
- W4207030291 cites W2513933594 @default.
- W4207030291 cites W2521785143 @default.
- W4207030291 cites W2523359503 @default.
- W4207030291 cites W2534647638 @default.
- W4207030291 cites W2559761446 @default.
- W4207030291 cites W2563543508 @default.
- W4207030291 cites W2582457239 @default.
- W4207030291 cites W2755712391 @default.
- W4207030291 cites W2770331714 @default.
- W4207030291 cites W2780710194 @default.
- W4207030291 cites W2789892520 @default.
- W4207030291 cites W2801294878 @default.
- W4207030291 cites W2804021909 @default.
- W4207030291 cites W2883834096 @default.
- W4207030291 cites W2884317633 @default.
- W4207030291 cites W2897987907 @default.
- W4207030291 cites W2901928825 @default.
- W4207030291 cites W2903358057 @default.
- W4207030291 cites W2908770791 @default.
- W4207030291 cites W2911392926 @default.
- W4207030291 cites W2921728659 @default.
- W4207030291 cites W2943032821 @default.
- W4207030291 cites W2944333148 @default.
- W4207030291 cites W2946683788 @default.
- W4207030291 cites W2968874575 @default.
- W4207030291 cites W2973118417 @default.
- W4207030291 cites W2973862854 @default.
- W4207030291 cites W2989454521 @default.