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The Human Cib1-Ever1-Ever2 Complex Governs Keratinocyte-Intrinsic Immunity to Β-Papillomaviruses Publisher Pubmed



De Jong SJ1 ; Crequer A1 ; Matos I2 ; Hum D1 ; Gunasekharan V3 ; Lorenzo L4, 5 ; Jabothanin F4, 5 ; Imahorn E6 ; Arias AA7, 8 ; Vahidnezhad H9, 10 ; Youssefian L9, 11 ; Markle JG1 ; Patin E12, 13, 14 ; Damico A1 Show All Authors
Authors
  1. De Jong SJ1
  2. Crequer A1
  3. Matos I2
  4. Hum D1
  5. Gunasekharan V3
  6. Lorenzo L4, 5
  7. Jabothanin F4, 5
  8. Imahorn E6
  9. Arias AA7, 8
  10. Vahidnezhad H9, 10
  11. Youssefian L9, 11
  12. Markle JG1
  13. Patin E12, 13, 14
  14. Damico A1
  15. Wang CQF15
  16. Full F16
  17. Ensser A16
  18. Leisner TM17
  19. Parise LV17
  20. Bouaziz M4, 5
  21. Maya NP18
  22. Cadena XR19
  23. Saka B20
  24. Saeidian AH9
  25. Aghazadeh N21
  26. Zeinali S10, 22
  27. Itin P6, 23
  28. Krueger JG15
  29. Laimins L3
  30. Abel L1, 4, 5
  31. Fuchs E2
  32. Uitto J9, 24
  33. Franco JL7
  34. Burger B6
  35. Orth G25
  36. Jouanguy E1, 4, 5
  37. Casanova JL1, 4, 5, 26, 27

Source: Journal of Experimental Medicine Published:2018


Abstract

Patients with epidermodysplasia verruciformis (EV) and biallelic null mutations of TMC6 (encoding EVER1) or TMC8 (EVER2) are selectively prone to disseminated skin lesions due to keratinocyte-tropic human β-papillomaviruses (β-HPVs), which lack E5 and E8. We describe EV patients homozygous for null mutations of the CIB1 gene encoding calcium- and integrin-binding protein-1 (CIB1). CIB1 is strongly expressed in the skin and cultured keratinocytes of controls but not in those of patients. CIB1 forms a complex with EVER1 and EVER2, and CIB1 proteins are not expressed in EVER1- or EVER2-deficient cells. The known functions of EVER1 and EVER2 in human keratinocytes are not dependent on CIB1, and CIB1 deficiency does not impair keratinocyte adhesion or migration. In keratinocytes, the CIB1 protein interacts with the HPV E5 and E8 proteins encoded by α-HPV16 and γ-HPV4, respectively, suggesting that this protein acts as a restriction factor against HPVs. Collectively, these findings suggest that the disruption of CIB1-EVER1-EVER2-dependent keratinocyte-intrinsic immunity underlies the selective susceptibility to β-HPVs of EV patients. © 2018 de Jong et al. This article is distributed under the terms of an Attribution-Noncommercial-Share Alike-No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms/). After six months it is available under a Creative Commons License
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