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Title: B cell development and activation defects resulting in xid-like immunodeficiency in BLNK/SLP-65-deficient mice
Authors: Xu, S.
Tan, J.E.-L.
Wong, E.P.-Y.
Manickam, A.
Ponniah, S. 
Lam, K.-P.
Keywords: Adaptor protein
B cell antigen receptor
CD5+ B cells
Gene targeting
Signal transduction
Issue Date: 2000
Citation: Xu, S.,Tan, J.E.-L.,Wong, E.P.-Y.,Manickam, A.,Ponniah, S.,Lam, K.-P. (2000). B cell development and activation defects resulting in xid-like immunodeficiency in BLNK/SLP-65-deficient mice. International Immunology 12 (3) : 397-404. ScholarBank@NUS Repository.
Abstract: Engagement of the a cell receptor (BCR) leads to the activation of tyrosine kinases and other signaling molecules that ultimately determine the type and magnitude of the B lymphocyte's cellular response. The adaptor protein BLNK/SLP-65 plays a pivotal role in BCR signal transduction by coupling Syk activation to downstream elements such as GrbS, phospholipase C-γ, Vav and Nck. We have generated BLNK(-/-) mice to determine the physiological role of this protein in B cell development and activation. BLNK(-/-) mice exhibit an incomplete block in a cell development with a severe inhibition of pro-B to pre-B cell differentiation. BLNK(-/-) slgM+ cells can develop, seed the peripheral lymphoid tissues and accumulate in numbers overtime. However, these mutant a cells failed to mature and are non-responsive to BCR cross-linking in terms of proliferation and upregulation of activation markers such as CD69 and CD86 (B7-2). In addition, the CD5+ subset of a cells is absent. The immune response to T cell-independent antigen but not T cell-dependent antigen is also impaired. Overall, the phenotype of BLNK(-/-) mice bears a striking resemblance to that of xid mice which is the murine model of human XLA that has a mutation in Bruton's tyrosine kinase. This raises the interesting possibility that mutation in BLNK/SLP-65 may be responsible for certain human immunodeficiencies.
Source Title: International Immunology
ISSN: 09538178
Appears in Collections:Staff Publications

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