title: Skewed distribution of circulating activated natural killer T (NKT) cells in patients with common variable immunodeficiency disorders (CVID).
abstract: Common variable immunodeficiency disorder (CVID) is the commonest cause of primary antibody failure in adults and children, and characterized clinically by recurrent bacterial infections and autoimmune manifestations. Several innate immune defects have been described in CVID, but no study has yet investigated the frequency, phenotype or function of the key regulatory cell population, natural killer T (NKT) cells. We measured the frequencies and subsets of NKT cells in patients with CVID and compared these to healthy controls. Our results show a skewing of NKT cell subsets, with CD4+ NKT cells at higher frequencies, and CD8+ NKT cells at lower frequencies. However, these cells were highly activated and expression CD161. The NKT cells had a higher expression of CCR5 and concomitantly expression of CCR5+CD69+CXCR6 suggesting a compensation of the remaining population of NKT cells for rapid effector action.
authors.affiliation: University of São Paulo, São Paulo, Brazil.
authors.forename: Karina I
authors.initials: KI
authors.lastname: Carvalho
authors.forename: Karina M
authors.initials: KM
authors.lastname: Melo
authors.forename: Fernanda R
authors.initials: FR
authors.lastname: Bruno
authors.forename: Jennifer E
authors.initials: JE
authors.lastname: Snyder-Cappione
authors.forename: Douglas F
authors.initials: DF
authors.lastname: Nixon
authors.forename: Beatriz T
authors.initials: BT
authors.lastname: Costa-Carvalho
authors.forename: Esper G
authors.initials: EG
authors.lastname: Kallas
mesh_terms: D000328:Adult; D018791:CD4 Lymphocyte Count; D016022:Case-Control Studies; D017074:Common Variable Immunodeficiency / Q000235:genetics / Q000276:immunology*; D005260:Female; D015870:Gene Expression; D006801:Humans; D008213:Lymphocyte Activation; D008297:Male; D055651:NK Cell Lectin-Like Receptor Subfamily B / Q000235:genetics / Q000276:immunology; D055611:Natural Killer T-Cells / Q000166:cytology / Q000276:immunology*; D019713:Receptors, CCR5 / Q000235:genetics / Q000276:immunology; D016176:T-Lymphocyte Subsets / Q000166:cytology / Q000276:immunology; D055815:Young Adult
chemical_list: D055651:NK Cell Lectin-Like Receptor Subfamily B; D019713:Receptors, CCR5