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Human Mcts1-Dependent Translation of Jak2 Is Essential for Ifn-Γ Immunity to Mycobacteria Publisher Pubmed



Bohlen J1, 2, 3, 4 ; Zhou Q5, 6 ; Philippot Q1, 2 ; Ogishi M5 ; Rinchai D5 ; Nieminen T7 ; Seyedpour S8, 9 ; Parvaneh N8, 10, 11 ; Rezaei N8, 12 ; Yazdanpanah N8, 12 ; Momenilandi M1, 2 ; Conil C1, 2 ; Neehus AL1, 2 ; Schmidt C5, 13 Show All Authors
Authors
  1. Bohlen J1, 2, 3, 4
  2. Zhou Q5, 6
  3. Philippot Q1, 2
  4. Ogishi M5
  5. Rinchai D5
  6. Nieminen T7
  7. Seyedpour S8, 9
  8. Parvaneh N8, 10, 11
  9. Rezaei N8, 12
  10. Yazdanpanah N8, 12
  11. Momenilandi M1, 2
  12. Conil C1, 2
  13. Neehus AL1, 2
  14. Schmidt C5, 13
  15. Arangofranco CA1, 2, 33
  16. Voyer TL1, 2
  17. Khan T14, 15, 16
  18. Yang R5
  19. Puchan J17
  20. Erazo L1, 2
  21. Roiuk M3, 4
  22. Vatovec T1, 2, 4
  23. Janda Z1, 2, 4
  24. Bagaric I1, 2, 4
  25. Materna M1, 2
  26. Gervais A1, 2
  27. Li H1, 2
  28. Rosain J1, 2
  29. Peel JN5
  30. Seeleuthner Y1, 2
  31. Han JE5
  32. Lhonneur AS18
  33. Moncadavelez M5
  34. Martinfernandez M19, 20, 21, 22, 23
  35. Horesh ME19, 20, 21, 22, 23
  36. Kochetkov T5
  37. Schmidt M24
  38. Alshehri MA25
  39. Salo E7
  40. Saxen H7
  41. Elghazali G26
  42. Yatim A5
  43. Soudee C1, 2
  44. Sallusto F17, 27
  45. Ensser A24
  46. Marr N14, 15
  47. Zhang P5
  48. Bogunovic D19, 20, 21, 22, 23
  49. Cobat A1, 2
  50. Shahrooei M28, 29
  51. Beziat V1, 2, 5
  52. Abel L1, 2, 5
  53. Wang X6
  54. Boissondupuis S1, 2, 5
  55. Teleman AA3, 4
  56. Bustamante J1, 2, 5, 30
  57. Zhang Q1, 2, 5
  58. Casanova JL1, 2, 5, 31, 32

Source: Cell Published:2023


Abstract

Human inherited disorders of interferon-gamma (IFN-γ) immunity underlie severe mycobacterial diseases. We report X-linked recessive MCTS1 deficiency in men with mycobacterial disease from kindreds of different ancestries (from China, Finland, Iran, and Saudi Arabia). Complete deficiency of this translation re-initiation factor impairs the translation of a subset of proteins, including the kinase JAK2 in all cell types tested, including T lymphocytes and phagocytes. JAK2 expression is sufficiently low to impair cellular responses to interleukin-23 (IL-23) and partially IL-12, but not other JAK2-dependent cytokines. Defective responses to IL-23 preferentially impair the production of IFN-γ by innate-like adaptive mucosal-associated invariant T cells (MAIT) and γδ T lymphocytes upon mycobacterial challenge. Surprisingly, the lack of MCTS1-dependent translation re-initiation and ribosome recycling seems to be otherwise physiologically redundant in these patients. These findings suggest that X-linked recessive human MCTS1 deficiency underlies isolated mycobacterial disease by impairing JAK2 translation in innate-like adaptive T lymphocytes, thereby impairing the IL-23-dependent induction of IFN-γ. © 2023 The Author(s)
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