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Science 3 December 1999:
Vol. 286. no. 5446, pp. 1954 - 1957
DOI: 10.1126/science.286.5446.1954
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Reports

An Essential Role for BLNK in Human B Cell Development

Yoshiyuki Minegishi, 1 Jurg Rohrer, 1 Elaine Coustan-Smith, 2 Howard M. Lederman, 3 Rajita Pappu, 4 Dario Campana, 25 Andrew C. Chan, 4 Mary Ellen Conley 15*

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.

1 Department of Immunology,
2 Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
3 Division of Pediatric Allergy and Immunology, Department of Pediatrics, Johns Hopkins Hospital, Baltimore, MD 21287, USA.
4 Center for Immunology, Division of Rheumatology, Departments of Medicine and Pathology, Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110, USA.
5 Department of Pediatrics, University of Tennessee, College of Medicine, Memphis, TN 38105, USA.
*   To whom correspondence should be addressed. E-mail: maryellen.conley{at}stjude.org

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