|
|
Blood, 1 April 2005, Vol. 105, No. 7, pp. 2933-2940.
Prepublished online as a Blood First Edition Paper on December 9, 2004; DOI 10.1182/blood-2004-09-3643.
 Previous Article | Next Article 
Submitted September 20, 2004
Accepted December 2, 2004
Lower levels of surface B-cell receptor expression in chronic lymphocytic leukemia are associated with glycosylation and folding defects of the µ and CD79a chains
Francoise Vuillier*, Gerard Dumas, Christian Magnac, Marie-Christine Prevost, Ana Ines Lalanne, Pablo Oppezzo, Evie Melanitou, Guillaume Dighiero, and Beatrice Payelle-Brogard
Unite d'Immuno-Hematologie et d'Immunopathologie, Institut Pasteur, Paris, France
Plate-forme de Microscopie Electronique, Institut Pasteur, Paris, France
* Corresponding author; email: vuillier{at}pasteur.fr.
Low levels of B-cell receptor (BCR) expression are the hallmark of tumoral B lymphocytes in B-cell chronic lymphocytic leukemia (B-CLL). These cells also respond inadequately to stimulation through the BCR. This receptor consists of a surface immunoglobulin associated with a CD79a/CD79b heterodimer. We previously showed that the intracellular synthesis of BCR components, from transcription onwards, is normal. Here, we investigated the glycosylation status and cellular localization of µ, CD79a and CD79b chains in 10 CLL patients differing in surface IgM expression. We reported a severe impairment of the glycosylation and folding of µ and CD79a. These defects were associated with the retention of both chains in the endoplasmic reticulum and lower levels of surface IgM expression. In contrast, no clear impairment of glycosylation and folding was observed for CD79b. No sequence defects were identified for BCR components and for the chaperone proteins involved in BCR folding processes. These data show, for the first time, that lower levels of BCR surface expression observed in CLL are accounted for by an impaired glycosylation and folding of the µ and CD79a chains.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
G. Cutrona, M. Colombo, S. Matis, M. Fabbi, M. Spriano, V. Callea, E. Vigna, M. Gentile, S. Zupo, N. Chiorazzi, et al.
Clonal heterogeneity in chronic lymphocytic leukemia cells: superior response to surface IgM cross-linking in CD38, ZAP-70-positive cells
Haematologica,
March 1, 2008;
93(3):
413 - 422.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Montel-Hagen, R. Vicente, and N. Taylor
Unveiling ZAP-70's plan B
Blood,
March 1, 2008;
111(5):
2501 - 2502.
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. Chen, L. Huynh, J. Apgar, L. Tang, L. Rassenti, A. Weiss, and T. J. Kipps
ZAP-70 enhances IgM signaling independent of its kinase activity in chronic lymphocytic leukemia
Blood,
March 1, 2008;
111(5):
2685 - 2692.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Ian Mockridge, K. N. Potter, I. Wheatley, L. A. Neville, G. Packham, and F. K. Stevenson
Reversible anergy of sIgM-mediated signaling in the two subsets of CLL defined by VH-gene mutational status
Blood,
May 15, 2007;
109(10):
4424 - 4431.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. N. Potter, C. I. Mockridge, L. Neville, I. Wheatley, M. Schenk, J. Orchard, A. S. Duncombe, G. Packham, and F. K. Stevenson
Structural and Functional Features of the B-Cell Receptor in IgG-Positive Chronic Lymphocytic Leukemia.
Clin. Cancer Res.,
March 15, 2006;
12(6):
1672 - 1679.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
