Article
Disruption of an antimycobacterial circuit between dendritic and helper T cells in human SPPL2a deficiency.
Nature immunology - 1 Sept 2018
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, Arlehamn Cecilia S Lindestam, 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
Abstract excerpt
Human inborn errors of IFN-γ immunity underlie mycobacterial diseases. We describe patients with Mycobacterium bovis (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-presenting cells. The CD74 NTF therefore accumulates in the HLA class II+ myeloid and lymphoid...
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