The murine VpreB1 and VpreB2 genes both encode a protein of the surrogate light chain and are co-expressed during B cell development

1996 ◽  
Vol 26 (4) ◽  
pp. 906-913 ◽  
Author(s):  
Jeanne L. Dul ◽  
Yair Argon ◽  
Thomas Winkler ◽  
Edwin ten Boekel ◽  
Fritz Melchers ◽  
...  
2000 ◽  
Vol 275 (40) ◽  
pp. 31134-31144 ◽  
Author(s):  
Kazuo Ohnishi ◽  
Takeyuki Shimizu ◽  
Hajime Karasuyama ◽  
Fritz Melchers

1993 ◽  
Vol 14 (2) ◽  
pp. 60-68 ◽  
Author(s):  
Fritz Melchers ◽  
Hajime Karasuyama ◽  
Dirk Haasner ◽  
Steven Bauer ◽  
Akira Kudo ◽  
...  

1996 ◽  
Vol 13 (4) ◽  
pp. 341-356 ◽  
Author(s):  
Antonius Rolink ◽  
Dirk Haasner ◽  
Fritz Melchers ◽  
Jan Andersson

1997 ◽  
Vol 815 (1 B-Lymphocytes) ◽  
pp. 193-196 ◽  
Author(s):  
CHRISTOPHE ARPIN ◽  
ODETTE de BOUTEILLER ◽  
DIANE RAZANAJAONA ◽  
FRANCINE BRIÈRE ◽  
JACQUES BANCHEREAU ◽  
...  

2007 ◽  
Vol 204 (9) ◽  
pp. 2047-2051 ◽  
Author(s):  
Simona Ferrari ◽  
Vassilios Lougaris ◽  
Stefano Caraffi ◽  
Roberta Zuntini ◽  
Jianying Yang ◽  
...  

Agammaglobulinemia is a rare primary immunodeficiency characterized by an early block of B cell development in the bone marrow, resulting in the absence of peripheral B cells and low/absent immunoglobulin serum levels. So far, mutations in Btk, μ heavy chain, surrogate light chain, Igα, and B cell linker have been found in 85–90% of patients with agammaglobulinemia. We report on the first patient with agammaglobulinemia caused by a homozygous nonsense mutation in Igβ, which is a transmembrane protein that associates with Igα as part of the preBCR complex. Transfection experiments using Drosophila melanogaster S2 Schneider cells showed that the mutant Igβ is no longer able to associate with Igα, and that assembly of the BCR complex on the cell surface is abrogated. The essential role of Igβ for human B cell development was further demonstrated by immunofluorescence analysis of the patient's bone marrow, which showed a complete block of B cell development at the pro-B to preB transition. These results indicate that mutations in Igβ can cause agammaglobulinemia in man.


1997 ◽  
Vol 185 (4) ◽  
pp. 609-620 ◽  
Author(s):  
Andrei Constantinescu ◽  
Mark S. Schlissel

The process of V(D)J recombination is crucial for regulating the development of B cells and for determining their eventual antigen specificity. Here we assess the developmental regulation of the V(D)J recombinase directly, by monitoring the double-stranded DNA breaks produced in the process of V(D)J recombination. This analysis provides a measure of recombinase activity at immunoglobulin heavy and light chain loci across defined developmental stages spanning the process of B cell development. We find that expression of a complete immunoglobulin heavy chain protein is accompanied by a drastic change in the targeting of V(D)J recombinase activity, from being predominantly active at the heavy chain locus in pro-B cells to being exclusively restricted to the light chain loci in pre-B cells. This switch in locus-specific recombinase activity results in allelic exclusion at the immunoglobulin heavy chain locus. Allelic exclusion is maintained by a different mechanism at the light chain locus. We find that immature, but not mature, B cells that already express a functional light chain protein can undergo continued light chain gene rearrangement, by replacement of the original rearrangement on the same allele. Finally, we find that the developmentally regulated targeting of V(D)J recombination is unaffected by enforced rapid transit through the cell cycle induced by an Eμ-myc transgene.


2017 ◽  
Vol 198 (4) ◽  
pp. 1565-1574 ◽  
Author(s):  
Carolina R. Batista ◽  
Stephen K. H. Li ◽  
Li S. Xu ◽  
Lauren A. Solomon ◽  
Rodney P. DeKoter

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