title: Intrinsic defects of B cell function in X-linked severe combined immunodeficiency.
abstract: The cytokine receptor common gamma chain mutation in X-linked SCID results in a failure of T and NK cell development and an as yet undefined defect of B cells. Using immunoglobulin isotype-specific reverse transcription-PCR we show that although hematopoietic stem cell transplantation restores a diverse repertoire of class-switched B cell clones, on further analysis these are almost all of donor origin. This suggests that host B cells, which predominate after unconditioned transplantation, are still defective even in the presence of normal T cells. These studies imply that effective humoral reconstitution can only be achieved by the engraftment of normal donor B cells.
authors.affiliation: Molecular Immunology Unit, Institute of Child Health, University College London, GB. h.white@ich.ucl.ac.uk
authors.forename: H
authors.initials: H
authors.lastname: White
authors.forename: A
authors.initials: A
authors.lastname: Thrasher
authors.forename: P
authors.initials: P
authors.lastname: Veys
authors.forename: C
authors.initials: C
authors.lastname: Kinnon
authors.forename: H B
authors.initials: HB
authors.lastname: Gaspar
mesh_terms: D001402:B-Lymphocytes / Q000276:immunology*; D001483:Base Sequence; D002675:Child, Preschool; D017931:DNA Primers / Q000235:genetics; D008040:Genetic Linkage; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007134:Immunoglobulin Switch Region / Q000235:genetics; D007142:Immunoglobulin gamma-Chains / Q000235:genetics; D007223:Infant; D016511:Severe Combined Immunodeficiency / Q000235:genetics* / Q000276:immunology* / Q000628:therapy; D019172:Transplantation Conditioning; D014960:X Chromosome / Q000235:genetics
chemical_list: D017931:DNA Primers; D007142:Immunoglobulin gamma-Chains