role of brutons tyrosine kinase in b cell development布鲁顿酪氨酸激酶b细胞发展的角色.pdf

Developmental Immunology, 2001, Vol. 8(3-4), pp. 171-181 (C) 2001 OPA (OverseasPublishers Association) Reprints available directly from the publisher N.V. Published by license under Photocopying permitted by license only the Harwood Academic Publishers imprint, part of The Gordon and Breach Publishing Group, member of the Taylor and Francis Group Role of Bruton’s Tyrosine Kinase in B Cell Development a ab* ALEX MAAS and RUDOLFW. HENDRIKS aDepartment ofCell Biology and Genetics andbDepartment ofImmunology, Erasmus University Rotterdam, POBox 1738, 3000 DR, Rotterdam, The Netherlands X-linked agammaglobulinemia (XLA) is one of the most frequent inherited immunodefi- ciency diseases in man and is characterized by an almost complete arrest of B cell differentia- tion at the pre-B cell stage. The gene defective in XLA encodes the cytoplasmic signaling molecule Bruton’s tyrosine kinase (Btk). Next to the CBA/N strain of mice, carrying a single amino acid substitution mutation in the Btk gene, which results in the X-linked immunodefi- ciency (xid) phenotype, additional mouse models have been developed to study the role of Btk in vivo. This review discusses the analyses of Btk null-mutants, obtained by gene target-