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Science 3 December 1999: Vol. 286. no. 5446, p. 1813 DOI: 10.1126/science.286.5446.1813j
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This Week in Science
Not all of the causes of genetic immunodeficiencies have been worked out. In cases in which B cells are lacking in the periphery, the Btk protein kinase signaling pathway is suspect, as it has turned out to be critical for B cell activation and development. Minegishi et al. (p. 1954) have characterized the defect in a patient that had normal Btk. The mutation responsible in this case was the BLNK adaptor protein, which coordinates signaling from the B cell antigen receptor by binding to various kinases, lipases, and adaptors. Pappu et al. (p. 1949) generated mice deficient in BLNK and report that these mice have the same defect--a block in the development of immature B cells from the pro-B to the pre-B stage that effectively prevents maturation of B cells and antibody production.
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Science. ISSN 0036-8075 (print), 1095-9203 (online)