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- 01H3A8ES0S9QKGDRSCB5RKPY83 classification A1.
- 01H3A8ES0S9QKGDRSCB5RKPY83 date "2023".
- 01H3A8ES0S9QKGDRSCB5RKPY83 language "eng".
- 01H3A8ES0S9QKGDRSCB5RKPY83 type journalArticle.
- 01H3A8ES0S9QKGDRSCB5RKPY83 hasPart 01H6P39AXKH26PW8EE62NBD2SG.pdf.
- 01H3A8ES0S9QKGDRSCB5RKPY83 subject "Biology and Life Sciences".
- 01H3A8ES0S9QKGDRSCB5RKPY83 subject "Medicine and Health Sciences".
- 01H3A8ES0S9QKGDRSCB5RKPY83 doi "10.3389/fimmu.2023.1188099".
- 01H3A8ES0S9QKGDRSCB5RKPY83 issn "1664-3224".
- 01H3A8ES0S9QKGDRSCB5RKPY83 volume "14".
- 01H3A8ES0S9QKGDRSCB5RKPY83 abstract "The Wiskott-Aldrich syndrome (WAS) is an X-linked primary immune deficiency caused by a mutation in the WAS gene. This leads to altered or absent WAS protein (WASp) expression and function resulting in thrombocytopenia, eczema, recurrent infections, and autoimmunity. In T cells, WASp is required for immune synapse formation. Patients with WAS show reduced numbers of peripheral blood T lymphocytes and an altered T-cell receptor repertoire. In vitro, their peripheral T cells show decreased proliferation and cytokine production upon aCD3/aCD28 stimulation. It is unclear whether these T-cell defects are acquired during peripheral activation or are, in part, generated during thymic development. Here, we assessed the role of WASp during T-cell differentiation using artificial thymic organoid cultures and in the thymus of humanized mice. Although CRISPR/Cas9 WAS knockout hematopoietic stem and progenitor cells (HSPCs) rearranged the T-cell receptor and differentiated to T-cell receptor (TCR)(+) CD4(+) CD8(+) double-positive (DP) cells similar to wild-type HSPCs, a partial defect in the generation of CD8 single-positive (SP) cells was observed, suggesting that WASp is involved in their positive selection. TCR repertoire analysis of the DP and CD8(+) SP population, however, showed a polyclonal repertoire with no bias toward autoreactivity. To our knowledge, this is the first study of the role of WASp in human T-cell differentiation and on TCR repertoire generation.".
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 1029F874-F0EE-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 2A59A78A-F0EE-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 31A7D41C-F0EE-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 3209D716-F0EE-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 36971884-F0EE-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 4C437678-1D89-11E3-818F-FB6410BDE39D.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 55ED044C-0F64-11E3-B31C-DCAC10BDE39D.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 9CDF3194-CDCD-11E9-886E-BDDF5607D3EF.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author 9FF28A18-FDEE-11E1-B8C9-1EA810BDE39D.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author CE3E88D4-FF80-11E1-9705-E1D010BDE39D.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author DE124A2A-3C5A-11E4-AAA0-50BEB4D1D7B1.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author F45EB9C2-F0ED-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author F4A869F0-F0ED-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author F552F726-F0ED-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author F5D916BC-F0ED-11E1-A9DE-61C894A0A6B4.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author urn:uuid:1bb80613-9979-435a-85f3-8b3b512106f8.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author urn:uuid:27a000d7-07c9-43f1-b57b-990f955dd839.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author urn:uuid:7db7273a-c081-49b5-bb1f-1ab92e582d7b.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author urn:uuid:856b9c5d-5b55-4370-9235-7f101fd05c26.
- 01H3A8ES0S9QKGDRSCB5RKPY83 author urn:uuid:fac2edb3-61a0-4c1c-8d50-c3cc379949e6.
- 01H3A8ES0S9QKGDRSCB5RKPY83 dateCreated "2023-06-19T16:40:22Z".
- 01H3A8ES0S9QKGDRSCB5RKPY83 dateModified "2024-12-12T18:00:45Z".
- 01H3A8ES0S9QKGDRSCB5RKPY83 name "The Wiskott–Aldrich syndrome protein is required for positive selection during T-cell lineage differentiation".
- 01H3A8ES0S9QKGDRSCB5RKPY83 pagination urn:uuid:9194b092-5906-4a92-af2b-084b3d1a150e.
- 01H3A8ES0S9QKGDRSCB5RKPY83 publisher urn:uuid:4dacc84f-0f69-4575-80ba-a4d4f82777f3.
- 01H3A8ES0S9QKGDRSCB5RKPY83 sameAs LU-01H3A8ES0S9QKGDRSCB5RKPY83.
- 01H3A8ES0S9QKGDRSCB5RKPY83 sourceOrganization urn:uuid:09662504-f168-49bb-9f10-3954090e3030.
- 01H3A8ES0S9QKGDRSCB5RKPY83 sourceOrganization urn:uuid:2decbd82-29fe-44e4-b50a-0f4e8f82abdc.
- 01H3A8ES0S9QKGDRSCB5RKPY83 sourceOrganization urn:uuid:66f070e0-f258-4590-9323-28ba20742bc0.
- 01H3A8ES0S9QKGDRSCB5RKPY83 sourceOrganization urn:uuid:698b29f4-b91d-4773-b6cc-2f19ea4620e1.
- 01H3A8ES0S9QKGDRSCB5RKPY83 sourceOrganization urn:uuid:79901f16-5bd6-47b7-b805-20b920823e08.
- 01H3A8ES0S9QKGDRSCB5RKPY83 type A1.