نتایج جستجو برای: rag 2 mutation

تعداد نتایج: 2746580  

2015
MARJORIE A. OETTINGER DAVID G. SCHATZ CAROLYN GoRKA DAVID BALTIMORE

The vast repertoire of immunoglobulins and T cell receptors is generated, in part, by V(D)J recombination, a series of genomic rearrangements that occur specifically in developing lymphocytes. The recombination activating gene, RAG-1, which is a gene expressed exclusively in maturing lymphoid cells, was previously isolated. RAG-1 inefficiently induced V(D)J recombinase activity when transfected...

2012
Agnieszka Laszkiewicz Lukasz Sniezewski Monika Kasztura Lukasz Bzdzion Malgorzata Cebrat Pawel Kisielow

The recombination-activating genes (RAG-1 and RAG-2) encode a V(D)J recombinase responsible for rearrangements of antigen-receptor genes during T and B cell development, and RAG expression is known to correlate strictly with the process of rearrangement. In contrast to RAG-1, the expression of RAG-2 was not previously detected during any other stage of lymphopoiesis or in any other normal tissu...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1994
W C Lin S Desiderio

The antigen receptors of B and T lymphocytes are encoded in multiple germ-line DNA segments that are joined during lymphocyte development. The recombination-activating proteins RAG-1 and RAG-2 are both essential for this process, termed V(D)J rearrangement. Phosphorylation of the RAG-2 protein at Thr-490 by one or more cyclin-dependent kinases is associated with its rapid degradation. In an imm...

Journal: :The Journal of Experimental Medicine 2000
David Nemazee Martin Weigert

B cell development is often portrayed as a series of decision points that expand an antigen-reactive cell to a clone producing a single antibody. This is hardly the case: B cell development is dependent on a series of error-prone, random rearrangement events that through ongoing diversification reach a compromise in which most cells are not au-toreactive (except in disease) and the majority of ...

2010
Caroline Montaudouin Laurent Boucontet Marie-Pierre Mailhé-Lembezat Maria-Encarnita Mariotti-Ferrandiz Anne Louise Adrien Six Antonio A. Freitas Sylvie Garcia

Recombination activating gene (RAG)-deficient TCR (T Cell Receptor) Tg (transgenic) mice are routinely used as sources of monoclonal T cells. We found that after the transfer of T cells from a RAG-2-deficient 5CC7 TCR Tg mice into allogeneic hosts we recovered a population of T cells expressing diverse alphabeta-TCRs. In fact, in the thymus and spleen of the 5CC7 RAG-2-deficient donor mice, we ...

Journal: :Journal of immunology 2002
Zhe-Xiong Jin Hiroyuki Kishi Xing-Cheng Wei Tadashi Matsuda Shigeru Saito Atsushi Muraguchi

The recombination-activating gene (RAG)-1 and RAG-2 are expressed specifically in immature lymphoid cells undergoing the recombination of Ag receptor genes. We studied the regulation of murine RAG-2 promoter and revealed that -41/-17 RAG-2 promoter region, which was indispensable for the RAG-2 promoter activity in B cell lines, contained binding sites for lymphoid enhancer-binding factor-1 (LEF...

Journal: :Developmental Immunology 1992
Jian Zhao Ursula Storb

Mice with the scid mutation have a defect in the V(D)J recombinase. In order to determine whether the SCID product is normally present in mature B cells that do not have the recombinase activity, scid pre-B cells were fused with myeloma cells. It was found that in the hybrid cells, a rearrangement test gene was correctly joined immediately after fusion. The same test gene was aberrantly rearran...

Journal: :Cell 1991
L M Carlson M A Oettinger D G Schatz E L Masteller E A Hurley W T McCormack D Baltimore C B Thompson

Chickens create their immunoglobulin (Ig) repertoires during B cell development in the bursa of Fabricius by intrachromosomal gene conversion. Recent evidence has suggested that Ig gene conversion may involve cis-acting DNA elements related to those involved in V(D)J recombination. Therefore, we have examined the potential role of the V(D)J recombination activating genes, RAG-1 and RAG-2, in re...

Journal: :Blood 2010
Suk See De Ravin Edward W Cowen Kol A Zarember Narda L Whiting-Theobald Douglas B Kuhns Netanya G Sandler Daniel C Douek Stefania Pittaluga Pietro L Poliani Yu Nee Lee Luigi D Notarangelo Lei Wang Frederick W Alt Elizabeth M Kang Joshua D Milner Julie E Niemela Mary Fontana-Penn Sara H Sinal Harry L Malech

Destructive midline granulomatous disease characterized by necrotizing granulomas of the head and neck is most commonly caused by Wegener granulomatosis, natural killer/T-cell lymphomas, cocaine abuse, or infections. An adolescent patient with myasthenia gravis treated with thymectomy subsequently developed extensive granulomatous destruction of midface structures, palate, nasal septum, airways...

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