Document Detail

Signal transduction through the B cell antigen receptor is normal in ataxia-telangiectasia B lymphocytes.
MedLine Citation:
PMID:  11733529     Owner:  NLM     Status:  MEDLINE    
The rare human genetic disorder ataxia-telangiectasia (A-T) has multiple consequences including a variable degree of immunodeficiency. Khanna and co-workers (Khanna, K. K., Yan, J., Watters, D., Hobson, K., Beamish, H., Spring, K., Shiloh, Y., Gatti, R. A., and Lavin, M. F. (1997) J. Biol. Chem. 272, 9489-9495) evaluated signaling in Epstein-Barr virus (EBV) immortalized A-T lymphoblastoid cell lines (LCLs), derived from the B cells of A-T patients. They showed that A-T lymphoblastoid cells lack signaling through the B cell antigen receptor and concluded that the fault in A-T encompasses intracellular signaling in B cells. However, it is established that EBV latent membrane protein 2A (LMP2A) blocks signaling in EBV-bearing cells by interaction with cellular tyrosine kinases. To test whether the reported fault in A-T B cells was not inherent in A-T but the result of influence of wild-type EBV, we derived A-T LCLs with wild-type or LMP2A-deleted EBV and studied signaling in these cells in response to cross-linking the B cell antigen receptor. We report that intracellular calcium mobilization and tyrosine phosphorylation in LMP2A-depleted LCLs derived from A-T patients is indistinguishable from that in LMP2A-depleted LCLs derived from normal controls. Further, signaling is blocked similarly in A-T and normal lymphoblastoid cells bearing wild-type EBV. In conclusion there is no evidence of any defect in B cell receptor signal transduction in A-T B cells.
Peter Speck; Masato Ikeda; Akiko Ikeda; Howard M Lederman; Richard Longnecker
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.     Date:  2001-12-03
Journal Detail:
Title:  The Journal of biological chemistry     Volume:  277     ISSN:  0021-9258     ISO Abbreviation:  J. Biol. Chem.     Publication Date:  2002 Feb 
Date Detail:
Created Date:  2002-02-04     Completed Date:  2002-03-05     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  2985121R     Medline TA:  J Biol Chem     Country:  United States    
Other Details:
Languages:  eng     Pagination:  4123-7     Citation Subset:  IM    
Department of Microbiology-Immunology, Northwestern University Medical School, Chicago, Illinois 60611, USA.
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MeSH Terms
Ataxia Telangiectasia / blood,  metabolism*
B-Lymphocytes / metabolism*
Calcium / metabolism
Cell Line, Transformed
Immunoglobulins / metabolism
Receptors, Antigen, B-Cell / metabolism*
Signal Transduction*
Tyrosine / metabolism
Viral Matrix Proteins / chemistry,  metabolism
Grant Support
Reg. No./Substance:
0/EBV-associated membrane antigen, Epstein-Barr virus; 0/Immunoglobulins; 0/Receptors, Antigen, B-Cell; 0/Viral Matrix Proteins; 55520-40-6/Tyrosine; 7440-70-2/Calcium

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