F
Françoise Le Deist
Researcher at Université de Montréal
Publications - 160
Citations - 23308
Françoise Le Deist is an academic researcher from Université de Montréal. The author has contributed to research in topics: T cell & Immunodeficiency. The author has an hindex of 65, co-authored 160 publications receiving 22286 citations. Previous affiliations of Françoise Le Deist include Centre Hospitalier Universitaire Sainte-Justine & Necker-Enfants Malades Hospital.
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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.
Journal ArticleDOI
A Serious Adverse Event after Successful Gene Therapy for X-Linked Severe Combined Immunodeficiency
Salima Hacein-Bey-Abina,Christof von Kalle,Manfred Schmidt,Françoise Le Deist,NM Wulffraat,Elisabeth McIntyre,Isabelle Radford,Jean-Luc Villeval,Christopher Fraser,Marina Cavazzana-Calvo,Alain Fischer +10 more
TL;DR: The sustained correction of X-linked severe combined immunodeficiency disease by ex vivo, retrovirally mediated transfer of the γc gene into CD34+ cells in four of five patients with the disease has been reported.
Journal ArticleDOI
Perforin Gene Defects in Familial Hemophagocytic Lymphohistiocytosis
Susan E. Stepp,Dufourcq-Lagelouse R,Françoise Le Deist,Françoise Le Deist,Sadhna Bhawan,Stéphanie Certain,Porunelloor A. Mathew,Jan-Inge Henter,Michael J. Bennett,Alain Fischer,Alain Fischer,Geneviève de Saint Basile,Vinay Kumar +12 more
TL;DR: In this paper, the coding regions of the perforin gene of eight unrelated 10q21-22-linked FHL patients revealed homozygous nonsense mutations in four patients and missense mutations in the other four patients.
Journal ArticleDOI
Sustained Correction of X-Linked Severe Combined Immunodeficiency by ex Vivo Gene Therapy
Salima Hacein-Bey-Abina,Françoise Le Deist,Frédérique Carlier,Cécile Bouneaud,Christophe Hue,Jean-Pierre de Villartay,Adrian J. Thrasher,NM Wulffraat,Ricardo U. Sorensen,Sophie Dupuis-Girod,Alain Fischer,E. Graham Davies,Wietse Kuis,Lilly Leiva,Marina Cavazzana-Calvo +14 more
TL;DR: Ex vivo gene therapy with gamma(c) can safely correct the immune deficiency of patients with X-linked severe combined immunodeficiency and allow patients to have a normal life.
Journal ArticleDOI
Artemis, a Novel DNA Double-Strand Break Repair/V(D)J Recombination Protein, Is Mutated in Human Severe Combined Immune Deficiency
Despina Moshous,Isabelle Callebaut,Régina de Chasseval,Barbara Corneo,Marina Cavazzana-Calvo,Françoise Le Deist,Ilhan Tezcan,Ozden Sanal,Yves Bertrand,N Philippe,Alain Fischer,Jean-Pierre de Villartay +11 more
TL;DR: The cloning of the gene encoding a novel protein involved in V(D)J recombination/DNA repair, Artemis, whose mutations cause human RS-SCID is described.