F
Françoise Selz
Researcher at Necker-Enfants Malades Hospital
Publications - 13
Citations - 3487
Françoise Selz is an academic researcher from Necker-Enfants Malades Hospital. The author has contributed to research in topics: T cell & Locus (genetics). The author has an hindex of 10, co-authored 13 publications receiving 3373 citations. Previous affiliations of Françoise Selz include University of Paris.
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Journal ArticleDOI
Gene therapy of human severe combined immunodeficiency (SCID)-X1 disease
Marina Cavazzana-Calvo,Hacein-Bey S,Geneviève de Saint Basile,Fabian Gross,Eric Yvon,Patrick Nusbaum,Françoise Selz,Christophe Hue,Stéphanie Certain,Jean-Laurent Casanova,Philippe Bousso,Françoise Le Deist,Alain Fischer +12 more
TL;DR: A gene therapy trial for SCID-X1 was initiated, based on the use of complementary DNA containing a defective gammac Moloney retrovirus-derived vector and ex vivo infection of CD34+ cells, which provided full correction of disease phenotype and clinical benefit.
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Autoimmune Lymphoproliferative Syndrome with Somatic Fas Mutations
Eliska Holzelova,Cédric Vonarbourg,Marie-Claude Stolzenberg,Peter D. Arkwright,Françoise Selz,Anne Marie Prieur,Stéphane Blanche,Jirina Bartunkova,Etienne Vilmer,Alain Fischer,Françoise Le Deist,Frédéric Rieux-Laucat +11 more
TL;DR: Somatic heterozygous mutations of Fas can cause a sporadic form of ALPS by allowing lymphoid precursors to resist the normal process of cell death.
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Defect in Rearrangement of the Most 5′ TCR–Jα Following Targeted Deletion of T Early α (TEA): Implications for TCR α Locus Accessibility
TL;DR: Results are consistent with TEA acting as a "rearrangement-focusing" element, targeting the primary waves of Vα–J α recombination to the most 5′ Jαs in an ongoing TCR–Jα rearrangement model.
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Inherited and Somatic CD3ζ Mutations in a Patient with T-Cell Deficiency
Frédéric Rieux-Laucat,Claire Hivroz,Annick Lim,Véronique Mateo,Isabelle Pellier,Françoise Selz,Alain Fischer,Françoise Le Deist +7 more
TL;DR: A four-month-old boy with primary immunodeficiency was found to have a homozygous germ-line mutation of the gene encoding the CD3zeta subunit of the T-cell receptor-CD3 complex, which is necessary for the development and function of T cells.
Journal ArticleDOI
Highly restricted human T cell repertoire in peripheral blood and tissue-infiltrating lymphocytes in Omenn's syndrome.
Frédéric Rieux-Laucat,Philippe Bahadoran,Nicole Brousse,Françoise Selz,A Fischer,F Le Deist,J P de Villartay +6 more
TL;DR: The results suggest that the oligoclonal expansion of T cells observed in Omenn's syndrome could be the consequence of autoimmune proliferation generated by a profound defect in lymphocyte development.