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

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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.
Lingua originaleEnglish
pagine (da-a)279-290
Numero di pagine12
RivistaCell
Volume72
DOI
Stato di pubblicazionePubblicato - 1993

Keywords

  • Agammaglobulinemia
  • Amino Acid Sequence
  • Animals
  • B-Lymphocytes
  • Blotting, Northern
  • Blotting, Southern
  • Cell Line
  • Chromosome Mapping
  • Cloning, Molecular
  • Cosmids
  • Cytoplasm
  • DNA
  • Heterozygote Detection
  • Humans
  • Hybrid Cells
  • Mice
  • Molecular Sequence Data
  • Protein-Tyrosine Kinases
  • RNA, Messenger
  • Sequence Homology, Amino Acid
  • Transcription, Genetic
  • Tumor Cells, Cultured
  • X Chromosome

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