Authors

Sarah Jill de Jong, Rockefeller University
Amandine Créquer, Rockefeller University
Irina Matos, Rockefeller University
David Hum, Rockefeller University
Vignesh Gunasekharan, Northwestern University
Lazaro Lorenzo, Necker Hospital for Sick Children; University Paris Descartes
Fabienne Jabot-Hanin, Necker Hospital for Sick Children; University Paris Descartes
Elias Imahorn, University of Basel
Andres A. Arias, University of Antioquia
Hassan Vahidnezhad, Thomas Jefferson University; Pasteur Institute of IranFollow
Leila Youssefian, Thomas Jefferson University; Tehran University of Medical SciencesFollow
Janet G. Markle, Rockefeller University
Etienne Patin, Pasteur Institute; National Center for Scientific Research;
Aurelia D'Amico, Rockefeller University
Claire Q.F. Wang, Rockefeller University
Florian Full, Friedrich‑Alexander‑University Erlangen‑Nuremberg
Armin Ensser, Friedrich‑Alexander‑University Erlangen‑Nuremberg
Tina M. Leisner, University of North Carolina at Chapel Hill
Leslie V. Parise, University of North Carolina at Chapel Hill
Matthieu Bouaziz, Necker Hospital for Sick Children; University Paris Descartes
Nataly Portilla Maya, Fundación Universitaria de Ciencias de la Salud
Xavier Rueda Cadena, National Cancer Institute, Bogota
Bayaki Saka, University of Lomé
Amir Hossein Saeidian, Thomas Jefferson UniversityFollow
Nessa Aghazadeh, Tehran University of Medical Sciences
Sirous Zeinali, Pasteur Institute of Iran; Kawsar Human Genetics Research Center
Peter Itin, University of Basel; University Hospital Basel
James G. Krueger, Rockefeller University
Lou Laimins, Northwestern University
Laurent Abel, Rockefeller University; Necker Hospital for Sick Children; University Paris Descartes
Elaine Fuchs, Rockefeller University
Jouni Uitto, Thomas Jefferson UniversityFollow
Jose Luis Franco, University of Antioquia
Bettina Burger, University of Basel
Gérard Orth, Pasteur Institute
Emmanuelle Jouanguy, Rockefeller University; Necker Hospital for Sick Children; University Paris Descartes
Jean-Laurent Casanova, Rockefeller University; Necker Hospital for Sick Children; University Paris Descartes; Howard Hughes Medical Institute

Document Type

Article

Publication Date

9-3-2018

Comments

This article has been peer reviewed. It is the author’s final published version in Journal of Experimental Medicine, Volume 215, Issue 9, September 2018, Pages 2289-2310.

The published version is available at https://doi.org/10.1084/jem.20170308. Copyright © De Jong et al.

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.

Creative Commons License

Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-Share Alike 4.0 License.

PubMed ID

30068544

Language

English

Available for download on Sunday, March 03, 2019

Included in

Dermatology Commons

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