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An Essential Role for BLNK in Human B Cell Development

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Science  03 Dec 1999:
Vol. 286, Issue 5446, pp. 1954-1957
DOI: 10.1126/science.286.5446.1954

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Abstract

The signal transduction events that control the progenitor B cell (pro-B cell) to precursor B cell (pre-B cell) transition have not been well delineated. In evaluating patients with absent B cells, a male with a homozygous splice defect in the cytoplasmic adapter protein BLNK (B cell linker protein) was identified. Although this patient had normal numbers of pro-B cells, he had no pre-B cells or mature B cells, indicating that BLNK plays a critical role in orchestrating the pro-B cell to pre-B cell transition. The immune system and overall growth and development were otherwise normal in this patient, suggesting that BLNK function is highly specific.

  • * To whom correspondence should be addressed. E-mail: maryellen.conley{at}stjude.org

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