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Article Dans Une Revue Journal of Clinical Immunology Année : 2013

EVER2 Deficiency is Associated with Mild T-cell Abnormalities

Résumé

Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by persistent flat warts or pityriasis versicolor-like lesions caused by betapapillomaviruses (EV-HPVs). Autosomal recessive EVER1 and EVER2 deficiencies account for EV in most patients. The mechanisms by which mutations in these partners of the Zinc transporter ZnT1 impair host defense against EV-HPVs are still poorly understood. Keratinocytes of EVER-deficient patients display an alteration of zinc homeostasis and an enhanced proliferative activity. Since EVER proteins are highly expressed in T lymphocytes, we aimed to assess the impact of EVER2 deficiency on T-cell development and function. We studied circulating lymphocyte populations in three adult EV patients sharing the same EVER2 mutation (T150fsX3). We found a normal count of CD4(+) and CD8(+) T cells and a normal proliferative capacity in response to anti-CD3 stimulation. However, we observed a significant increase of memory CD4(+) and effector memory CD8(+) T cells, a bias of the TCR Vαβ and Vγδ repertoires and an increase of skin-homing CD4(+) T-cell subsets. Our findings suggest that EVER2-deficient patients display mild T-cell abnormalities. It remains unclear whether these abnormalities result from EVER deficiency, chronic EV-HPV infection, or both.

Dates et versions

hal-03691617 , version 1 (09-06-2022)

Identifiants

Citer

Amandine Crequer, Capucine Picard, Vincent Pedergnana, Annick Lim, Shen-Ying Zhang, et al.. EVER2 Deficiency is Associated with Mild T-cell Abnormalities. Journal of Clinical Immunology, 2013, 33 (1), pp.14-21. ⟨10.1007/s10875-012-9749-1⟩. ⟨hal-03691617⟩

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