期刊
JOURNAL OF EXPERIMENTAL MEDICINE
卷 200, 期 7, 页码 927-934出版社
ROCKEFELLER UNIV PRESS
DOI: 10.1084/jem.20040920
关键词
XLA; Bruton's tyrosine kinase; B lymphocytes; tolerance; autoantibody
Most polyreactive and antinuclear antibodies are removed from the human antibody repertoire during B cell development. To elucidate how B cell receptor (BCR) signaling may regulate human B cell tolerance, we tested the specificity of recombinant antibodies from single peripheral B cells isolated from patients suffering from X-linked agammaglobulinemia (XLA). These patients carry mutations in the Bruton's tyrosine kinase (BTK) gene that encode an essential BCR signaling component. We find that in the absence of Btk, peripheral B cells show a distinct antibody repertoire consistent with extensive secondary V(D)J recombination. Nevertheless, XLA B cells are enriched in autoreactive clones. Our results demonstrate that Btk is essential in regulating thresholds for human B cell tolerance.
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