Intrinsic defects of B cell function in X-linked severe combined immunodeficiency.
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.
White
H
H
Molecular Immunology Unit, Institute of Child Health, University College London, GB. h.white@ich.ucl.ac.uk
Thrasher
A
A
Veys
P
P
Kinnon
C
C
Gaspar
H B
HB
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
D017931:DNA Primers; D007142:Immunoglobulin gamma-Chains