title: Progress in primary immunodeficiency.
abstract: Clinically, primary immunodeficiencies can be grouped into several well-defined syndromes. This consistent clinical picture, however, belies the enormous complexity of lymphocyte maturation and activation, and it has long been suspected that numerous distinct underlying defects give rise to primary immunodeficiencies. At a recent workshop, the molecular and genetic characterization of a handful of such defects provided tacit support for such suspicions.
authors.affiliation: Dept of Pediatrics, Hokkaido University School of Medicine, Sapporo, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Matsumoto
authors.forename: Y
authors.initials: Y
authors.lastname: Sakiyama
authors.forename: T
authors.initials: T
authors.lastname: Ariga
authors.forename: R
authors.initials: R
authors.lastname: Gallagher
authors.forename: Y
authors.initials: Y
authors.lastname: Taguchi
mesh_terms: D000243:Adenosine Deaminase / Q000172:deficiency / Q000235:genetics; D015316:Genetic Therapy; D006680:HLA Antigens; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000209:etiology* / Q000628:therapy; D016511:Severe Combined Immunodeficiency / Q000209:etiology / Q000628:therapy; D014923:Wiskott-Aldrich Syndrome / Q000209:etiology
chemical_list: D006680:HLA Antigens; D000243:Adenosine Deaminase