Human B-Cell Development in a Mouse Environment

2014 ◽  
pp. 141-152
Author(s):  
Julie Lang ◽  
Roberta Pelanda
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.


2016 ◽  
Vol 7 ◽  
Author(s):  
Victoria G. Martin ◽  
Yu-Chang Bryan Wu ◽  
Catherine L. Townsend ◽  
Grace H. C. Lu ◽  
Joselli Silva O’Hare ◽  
...  

1986 ◽  
Vol 5 (8) ◽  
pp. 1783-1790 ◽  
Author(s):  
A. Tulp ◽  
M. Barnhoorn ◽  
E. Bause ◽  
H. Ploegh

1996 ◽  
Vol 183 (6) ◽  
pp. 2693-2698 ◽  
Author(s):  
E Sanz ◽  
A de la Hera

Vpre-B and lambda 5 genes, respectively, encode V-like and C-like domains of a surrogate immunoglobulin light chain (psi L). Such psi L complex is expressed in early progenitor B (pro-B) cells, before conventional immunoglobulin heavy (microH) and light (L) chains are produced. We raised a wide panel of monoclonal antibodies (mAbs) against soluble recombinant Vpre-B proteins to study early events in human B cell development. One of these antibodies, B-MAD688, labeled surrogate Ig-complexes on the surface of microH- pro-B cell lines and normal bone marrow cells in immunofluorescence assays. Immunoprecipitations using surface-labeled pro-B cells and B-MAD688 mAb indicated that human psi L is associated with high molecular weight components homologous to the surrogate heavy (psi H) chains described in mouse. Using B-MAD688 and SLC2 mAbs, we were able to distinguish between psi H psi L and microH psi L complexes on the surface of human pro-B and later precursor, pre-B, cells. The finding of psi H psi L complexes in mouse and man lead us to hypothesize a role for psi H-containing receptors in B cell development.


2013 ◽  
Vol 131 (6) ◽  
pp. 1695-1699.e9 ◽  
Author(s):  
Anne-Kathrin Kienzler ◽  
Marta Rizzi ◽  
Maike Reith ◽  
Stephen L. Nutt ◽  
Hermann Eibel

2010 ◽  
Vol 31 (2) ◽  
pp. 253-264 ◽  
Author(s):  
Bongkum Choi ◽  
Eunyoung Chun ◽  
Miyoung Kim ◽  
Seong-Tae Kim ◽  
Keejung Yoon ◽  
...  

2006 ◽  
Vol 34 (8) ◽  
pp. 1093-1100 ◽  
Author(s):  
Marek Honczarenko ◽  
Aleksandra M. Glodek ◽  
Marcin Swierkowski ◽  
Il-Kang Na ◽  
Leslie E. Silberstein

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