The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses

Publication Date:
2018-09-04
Publisher:
Rockefeller University Press
Print ISSN:
0022-1007
Electronic ISSN:
1540-9538
Topics:
Medicine
Keywords:
Immunodeficiency, Infectious Disease and Host Defense, Human Disease Genetics
Published by:
http://www.rupress.org/
_version_ 1836399042397995008
autor de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
beschreibung 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 -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.
citation_standardnr 6327529
datenlieferant ipn_articles
feed_id 96
feed_publisher Rockefeller University Press
feed_publisher_url http://www.rupress.org/
insertion_date 2018-09-04
journaleissn 1540-9538
journalissn 0022-1007
publikationsjahr_anzeige 2018
publikationsjahr_facette 2018
publikationsjahr_intervall 7984:2015-2019
publikationsjahr_sort 2018
publisher Rockefeller University Press
quelle Journal of Experimental Medicine
relation http://jem.rupress.org/cgi/content/short/215/9/2289?rss=1
schlagwort Immunodeficiency, Infectious Disease and Host Defense, Human Disease Genetics
search_space articles
shingle_author_1 de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
shingle_author_2 de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
shingle_author_3 de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
shingle_author_4 de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
shingle_catch_all_1 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
Immunodeficiency, Infectious Disease and Host Defense, Human Disease Genetics
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 -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.
de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
Rockefeller University Press
0022-1007
00221007
1540-9538
15409538
shingle_catch_all_2 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
Immunodeficiency, Infectious Disease and Host Defense, Human Disease Genetics
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 -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.
de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
Rockefeller University Press
0022-1007
00221007
1540-9538
15409538
shingle_catch_all_3 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
Immunodeficiency, Infectious Disease and Host Defense, Human Disease Genetics
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 -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.
de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
Rockefeller University Press
0022-1007
00221007
1540-9538
15409538
shingle_catch_all_4 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
Immunodeficiency, Infectious Disease and Host Defense, Human Disease Genetics
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 -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.
de Jong, S. J., Crequer, A., Matos, I., Hum, D., Gunasekharan, V., Lorenzo, L., Jabot-Hanin, F., Imahorn, E., Arias, A. A., Vahidnezhad, H., Youssefian, L., Markle, J. G., Patin, E., DAmico, A., Wang, C. Q. F., Full, F., Ensser, A., Leisner, T. M., Parise, L. V., Bouaziz, M., Maya, N. P., Cadena, X. R., Saka, B., Saeidian, A. H., Aghazadeh, N., Zeinali, S., Itin, P., Krueger, J. G., Laimins, L., Abel, L., Fuchs, E., Uitto, J., Franco, J. L., Burger, B., Orth, G., Jouanguy, E., Casanova, J.-L.
Rockefeller University Press
0022-1007
00221007
1540-9538
15409538
shingle_title_1 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
shingle_title_2 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
shingle_title_3 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
shingle_title_4 The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
timestamp 2025-06-30T23:36:41.168Z
titel The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
titel_suche The human CIB1-EVER1-EVER2 complex governs keratinocyte-intrinsic immunity to {beta}-papillomaviruses
topic WW-YZ
uid ipn_articles_6327529