Disruption of an anti-mycobacterial circuit between dendritic and Th cells in human SPPL2a deficiency

Human inborn errors of IFN-γ immunity underlie mycobacterial diseases. We describe patients with BCG disease who are homozygous for loss-of-function mutations of SPPL2A. This gene encodes a transmembrane protease that degrades the N-terminal fragment (NTF) of CD74 (HLA invariant chain) in antigen-pr...

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Autores principales: Kong, Xiao-Fei, Martinez-Barricarte, Ruben, Kennedy, James, Mele, Federico, Lazarov, Tomi, Deenick, Elissa K, Ma, Cindy S, Breton, Gaëlle, Lucero, Kimberly B, Langlais, David, Bousfiha, Aziz, Aytekin, Caner, Markle, Janet, Trouillet, Céline, Jabot-Hanin, Fabienne, Lindestam Arlehamn, Cecilia S., Rao, Geetha, Picard, Capucine, Lasseau, Théo, Latorre, Daniela, Hambleton, Sophie, Deswarte, Caroline, Itan, Yuval, Abarca, Katia, Moraes-Vasconcelos, Dewton, Ailal, Fatima, Ikinciogullari, Aydan, Dogu, Figen, Benhsaien, Ibtihal, Sette, Alessandro, Abel, Laurent, Boisson-Dupuis, Stéphanie, Schröder, Bernd, Nussenzweig, Michel C., Liu, Kang, Geissmann, Frédéric, Tangye, Stuart G., Gros, Philippe, Sallusto, Federica, Bustamante, Jacinta, Casanova, Jean-Laurent
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6130844/
https://www.ncbi.nlm.nih.gov/pubmed/30127434
http://dx.doi.org/10.1038/s41590-018-0178-z

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