Cernunnos Deficiency Reduces Thymocyte Life Span and Alters the T Cell Repertoire in Mice and Humans
Copyright policy )Cernunnos Deficiency Reduces Thymocyte Life Span and Alters the T Cell Repertoire in Mice and Humans
Cernunnos is a DNA repair factor of the nonhomologous end-joining machinery. Its deficiency in humans causes radiosensitive severe combined immune deficiency (SCID) with microcephaly, characterized in part by a profound lymphopenia. In contrast to the human condition, the immune system of Cernunnos knockout (KO) mice is not overwhelmingly affected. In particular, Cernunnos is dispensable during V(D)J recombination in lymphoid cells. Nevertheless, the viability of thymocytes is reduced in Cernunnos KO mice, owing to the chronic activation of a P53-dependent DNA damage response. This translates into a qualitative alteration of the T cell repertoire to one in which the most distal Vα and Jα segments are missing. This results in the contraction of discrete T cell populations, such as invariant natural killer T (iNKT) and mucosa-associated invariant T (MAIT) cells, in both humans and mice.
- Institut Pasteur France
- University of Paris France
- Inserm France
- French Institute of Health and Medical Research France
- Necker-Enfants Malades Hospital France
Mice, Knockout, Thymocytes, Base Sequence, DNA Repair, Cell Survival, T-Lymphocytes, Molecular Sequence Data, V(D)J Recombination, DNA-Binding Proteins, Gene Knockout Techniques, Mice, DNA Repair Enzymes, Animals, Humans, Lymphocyte Count, Tumor Suppressor Protein p53, Cell Proliferation
Mice, Knockout, Thymocytes, Base Sequence, DNA Repair, Cell Survival, T-Lymphocytes, Molecular Sequence Data, V(D)J Recombination, DNA-Binding Proteins, Gene Knockout Techniques, Mice, DNA Repair Enzymes, Animals, Humans, Lymphocyte Count, Tumor Suppressor Protein p53, Cell Proliferation
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