Localization, interaction, and RNA binding properties of the V(D)J recombination-activating proteins RAG1 and RAG2
Eugenia Spanopoulou,Patricia Cortes,Charles Shih,Chun-Ming Huang,Daniel P. Silver,Pamela Svec,David Baltimore +6 more
TLDR
RAG1 appears to have a binary structure, each half containing multiple regions that can act as NLSs, binding sites for the SRP1/Rch1 family, and RNA binding domains, indicating that RAG1 has affinity for RNA or ssDNA.About:
This article is published in Immunity.The article was published on 1995-12-01 and is currently open access. It has received 100 citations till now. The article focuses on the topics: RNA-binding protein & RNA.read more
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The RAG proteins and V(D)J recombination: complexes, ends, and transposition.
TL;DR: A model of the RAG protein active site is suggested in which two divalent metal ions serve alternating and opposite roles as activators of attacking hydroxyl groups and stabilizers of oxyanion leaving groups.
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V(D)J recombination: mechanisms of initiation.
TL;DR: This review considers the functional significance of RAG-mediated histone recognition and ubiquitin ligase activities, and the role played by RAG in ensuring proper repair of DNA breaks made during V(D)J recombination, and proposes a model for the formation of R AG-DNA complexes.
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Partial V(D)J Recombination Activity Leads to Omenn Syndrome
Anna Villa,Sandro Santagata,Fabio Bozzi,Silvia Giliani,Annalisa Frattini,Luisa Imberti,Luisa Benerini Gatta,Hans D. Ochs,Klaus Schwarz,Luigi D. Notarangelo,Paolo Vezzoni,Eugenia Spanopoulou +11 more
TL;DR: It is reported here that patients with Omenn syndrome, a severe immunodeficiency characterized by the presence of activated, anergic, oligoclonal T cells, hypereosinophilia, and high IgE levels, bear missense mutations in either the Rag-1 or Rag-2 genes that result in partial activity of the two proteins.
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Neoteny in Lymphocytes: Rag1 and Rag2 Expression in Germinal Center B Cells
TL;DR: Abundant RAG1 and RAG2 protein and messenger RNA was detected in the activated B cells that populate murine splenic and Peyer's patch germinal centers, raising the possibility that antigen-dependent secondary V(D)J rearrangements modify the peripheral antibody repertoire.
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Reexpression of RAG-1 and RAG-2 Genes in Activated Mature Mouse B cells
Masaki Hikida,Masaharu Mori,Toshiyuki Takai,Ken Ichi Tomochika,Kiyohiro Hamatani,Hitoshi Ohmori +5 more
TL;DR: RAG-1 and RAG-2 proteins could be detected by immunofluorescence microscopy in the nuclei of B cells cultured in vitro and in the germinal centers of draining lymph nodes, suggesting that RAG gene products play a heretofore unseen role in mature B cells.
References
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Somatic generation of antibody diversity
TL;DR: In the genome of a germ-line cell, the genetic information for an immunoglobulin polypeptide chain is contained in multiple gene segments scattered along a chromosome which are assembled by recombination which leads to the formation of a complete gene.
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RAG-1-deficient mice have no mature B and T lymphocytes
Peter Mombaerts,John Iacomini,Randall S. Johnson,Karl Herrup,Susumu Tonegawa,Virginia E. Papaioannou +5 more
TL;DR: The introduction of a mutation in RAG-1 into the germline of mice via gene targeting in embryonic stem cells is described and it is shown that this mutation either activates or catalyzes the V(D)J recombination reaction of immunoglobulin and T cell receptor genes.
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RAG-2-deficient mice lack mature lymphocytes owing to inability to initiate V(D)J rearrangement
Yoichi Shinkai,Gary Rathbun,Kong-Peng Lam,E. M. Oltz,Valerie Stewart,Monica Mendelsohn,Jean Charron,Milton Datta,Faith Young,Alan M. Stall,Frederick W. Alt +10 more
TL;DR: Loss of RAG-2 function in vivo results in total inability to initiate V(D)J rearrangement, leading to a novel severe combined immune deficient (SCID) phenotype.
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Nuclear targeting sequences--a consensus?
TL;DR: It is suggested in this review that, despite this diversity of nuclear targeting sequences, a consensus bipartite motif can be identified.
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Conserved structures and diversity of functions of RNA-binding proteins.
TL;DR: The major RNA-binding motifs are described and examples of how they may function are given.