Article
MST1 mutations in autosomal recessive primary immunodeficiency characterized by defective naive T-cell survival.
Blood - 12 Apr 2012
Nehme Nadine T, Schmid Jana Pachlopnik, Debeurme Franck, André-Schmutz Isabelle, Lim Annick, Nitschke Patrick, Rieux-Laucat Frédéric, Lutz Patrick, Picard Capucine, Mahlaoui Nizar, Fischer Alain, de Saint Basile Geneviève
Abstract excerpt
The molecular mechanisms that underlie T-cell quiescence are poorly understood. In the present study, we report a primary immunodeficiency phenotype associated with MST1 deficiency and primarily characterized by a progressive loss of naive T cells. The in vivo consequences include recurrent bacterial and viral infections and autoimmune manifestations. MST1-deficient T cells poorly expressed the transcription...
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