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https://www.selleckchem.com/products/ms-275.html RESULTS We identified a homozygous frameshift mutation in TNFSF13, the gene encoding APRIL, in the patient. APRIL mRNA and protein were completely absent in the monocytes and iPS-moDCs of the patient. In contrast to previous animal model studies, the patient showed hypogammaglobulinemia with markedly reduced plasmacytes in peripheral blood and clearly increased circulating marginal zone B cells. Although iPS-moDC-induced in vitro plasmacyte differentiation was reduced in the patient, recombinant APRIL supplementation corrected this abnormality. CONCLUSION The first APRIL deficiency in an adult common variable immunodeficiency patient revealed the role of APRIL in life-long maintenance of plasmacytes and immunoglobulin production in humans. BACKGROUND Whether microbiome characteristics of induced sputum or oral samples demonstrate unique relationships to features of atopy or mild asthma in adults is unknown. OBJECTIVE To determine sputum and oral microbiota relationships to clinical or immunologic features in mild atopic asthma and the impact on the microbiota of inhaled corticosteroid (ICS) treatment administered to ICS-naïve asthmatic subjects. METHODS Bacterial microbiota profiles were analyzed in induced sputum and oral wash samples from 32 subjects with mild atopic asthma before and after inhaled fluticasone treatment, 18 atopic non-asthmatic subjects, and 16 non-atopic healthy subjects in a multicenter study (NCT01537133). Associations with clinical and immunologic features were examined, including markers of atopy, type 2 inflammation, immune cell populations and cytokines. RESULTS Sputum bacterial burden inversely associated with bronchial expression of type 2 (T2)-related genes. Differences in specific sputum microbiota also associated with T2-low asthma phenotype, a subgroup of whom displayed elevations in lung inflammatory mediators and reduced sputum bacterial diversity. Differences in specific oral microbi

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