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      Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia.

      Cell

      X Chromosome, Agammaglobulinemia, enzymology, genetics, immunology, Amino Acid Sequence, Animals, B-Lymphocytes, Blotting, Northern, Blotting, Southern, Cell Line, Chromosome Mapping, Cloning, Molecular, Cosmids, Cytoplasm, DNA, isolation & purification, Heterozygote Detection, Humans, Hybrid Cells, cytology, Mice, Molecular Sequence Data, Protein-Tyrosine Kinases, deficiency, RNA, Messenger, metabolism, Sequence Homology, Amino Acid, Transcription, Genetic, Tumor Cells, Cultured

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          Abstract

          We describe a novel cytoplasmic tyrosine kinase, termed BPK (B cell progenitor kinase), which is expressed in all stages of the B lineage and in myeloid cells. BPK has classic SH1, SH2, and SH3 domains, but lacks myristylation signals and a regulatory phosphorylation site corresponding to tyrosine 527 of c-src. BPK has a long, basic amino-terminal region upstream of the SH3 domain. BPK was evaluated as a candidate for human X-linked agammaglobulinemia (XLA), an inherited immunodeficiency characterized by a severe deficit of B and plasma cells and profound hypogammaglobulinemia. BPK mapped to within 100 kb of a probe defining the polymorphism most closely linked to XLA at DXS178. Reduction in or the absence of BPK mRNA, protein expression, and kinase activity was observed in XLA pre-B and B cell lines. BPK is likely the XLA gene and functions in pathways critical to B cell expansion.

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          8425221

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