Omenn syndrome: a disorder of Rag1 and Rag2 genes. (Q33607425)

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5 December 2019

review article

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title

Omenn syndrome: a disorder of Rag1 and Rag2 genes (English)

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5 December 2019

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5 December 2019

A Villa

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5 December 2019

S Santagata

series ordinal

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5 December 2019

L Imberti

series ordinal

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5 December 2019

L D Notarangelo

series ordinal

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5 December 2019

publication date

1 March 1999

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Journal of Clinical Immunology

5 December 2019

published in

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5 December 2019

volume

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5 December 2019

issue

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5 December 2019

page(s)

87-97

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Identification by HLA typing of intrauterine-derived maternal T cells in four patients with severe combined immunodeficiency

5 December 2019

cites work

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12 December 2020

Nonsense mutation at Tyr-4046 in the DNA-dependent protein kinase catalytic subunit of severe combined immune deficiency mice

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12 December 2020

Combined immunodeficiency and reticuloendotheliosis with eosinophilia

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12 December 2020

The mechanism of V(D)J joining: lessons from molecular, immunological, and comparative analyses

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12 December 2020

The V(D)J recombination activating gene, RAG-1.

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12 December 2020

DNA double-strand break repair and V(D)J recombination: involvement of DNA-PK.

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12 December 2020

RAG1 and RAG2 form a stable postcleavage synaptic complex with DNA containing signal ends in V(D)J recombination

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12 December 2020

Initiation of V(D)J recombination in a cell-free system.

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12 December 2020

Development of TH1 CD4+ T cells through IL-12 produced by Listeria-induced macrophages

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12 December 2020

Activity of DNA ligase IV stimulated by complex formation with XRCC4 protein in mammalian cells

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12 December 2020

The cell cycle and V(D)J recombination.

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12 December 2020

The Lymph Node Pathology of Omennʼs Syndrome

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12 December 2020

Genetically resistant mice lacking interleukin-12 are susceptible to infection with Leishmania major and mount a polarized Th2 cell response.

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12 December 2020

Biochemical and genetic defects in the DNA-dependent protein kinase in murine scid lymphocytes

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12 December 2020

Neoteny in lymphocytes: Rag1 and Rag2 expression in germinal center B cells

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12 December 2020

Defective DNA-dependent protein kinase activity is linked to V(D)J recombination and DNA repair defects associated with the murine scid mutation.

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12 December 2020

Hairpin coding end opening is mediated by RAG1 and RAG2 proteins

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12 December 2020

Sequence of the spacer in the recombination signal sequence affects V(D)J rearrangement frequency and correlates with nonrandom Vkappa usage in vivo

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12 December 2020

Stimulation of V(D)J cleavage by high mobility group proteins

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12 December 2020

Impaired IL-12 responses and enhanced development of Th2 cells in Stat4-deficient mice

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12 December 2020

Increased radiosensitivity of granulocyte macrophage colony-forming units and skin fibroblasts in human autosomal recessive severe combined immunodeficiency

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12 December 2020

RAG mutations in human B cell-negative SCID.

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12 December 2020

Increased frequency of N-region insertion in a murine pre-B-cell line infected with a terminal deoxynucleotidyl transferase retroviral expression vector

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12 December 2020

Targeted disruption of the catalytic subunit of the DNA-PK gene in mice confers severe combined immunodeficiency and radiosensitivity

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12 December 2020

RAG-2-deficient mice lack mature lymphocytes owing to inability to initiate V(D)J rearrangement

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12 December 2020

[Severe combined immune deficiency with hypereosinophilia. Immunologic study of 5 cases]

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12 December 2020

Identification of a nonsense mutation in the carboxyl-terminal region of DNA-dependent protein kinase catalytic subunit in the scid mouse

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12 December 2020

Novel T-lymphocyte population in combined immunodeficiency with features of graft-versus-host disease

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12 December 2020

Somatic generation of antibody diversity

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12 December 2020

Expression and V(D)J recombination activity of mutated RAG-1 proteins

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12 December 2020

Definition of a large region of RAG1 that is important for coimmunoprecipitation of RAG2

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12 December 2020

V(D)J recombination: modulation of RAG1 and RAG2 cleavage activity on 12/23 substrates by whole cell extract and DNA-bending proteins

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12 December 2020

Restricted heterogeneity of T lymphocytes in combined immunodeficiency with hypereosinophilia (Omenn's syndrome).

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12 December 2020

Mechanism of V(D)J recombination

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12 December 2020

CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: evidence for a T helper 2-mediated condition

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12 December 2020

Transposition mediated by RAG1 and RAG2 and its implications for the evolution of the immune system

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12 December 2020

Treatment of Omenn syndrome by bone marrow transplantation

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12 December 2020

In vitro cell death of activated lymphocytes in Omenn's syndrome

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12 December 2020

A human severe combined immunodeficiency (SCID) condition with increased sensitivity to ionizing radiations and impaired V(D)J rearrangements defines a new DNA recombination/repair deficiency

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12 December 2020

Primordial emergence of the recombination activating gene 1 (RAG1): sequence of the complete shark gene indicates homology to microbial integrases

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12 December 2020

Reexpression of RAG-1 and RAG-2 genes in activated mature mouse B cells

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12 December 2020

Conservation of sequence in recombination signal sequence spacers

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12 December 2020

Oligoclonal expansion of CD45RO+ T lymphocytes in Omenn syndrome

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12 December 2020

A stable RAG1-RAG2-DNA complex that is active in V(D)J cleavage

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12 December 2020

The homeodomain region of Rag-1 reveals the parallel mechanisms of bacterial and V(D)J recombination

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12 December 2020

Localization, interaction, and RNA binding properties of the V(D)J recombination-activating proteins RAG1 and RAG2.

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12 December 2020

Cleavage at a V(D)J recombination signal requires only RAG1 and RAG2 proteins and occurs in two steps

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12 December 2020

Growth retardation and leaky SCID phenotype of Ku70-deficient mice.

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12 December 2020

The recombination activating genes, RAG 1 and RAG 2, are on chromosome 11p in humans and chromosome 2p in mice

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12 December 2020

T helper type 2-like cells and therapeutic effects of interferon-gamma in combined immunodeficiency with hypereosinophilia (Omenn's syndrome)

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12 December 2020

IL-12-deficient mice are defective in IFN gamma production and type 1 cytokine responses

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12 December 2020

A Basic Motif in the N-Terminal Region of RAG1 Enhances V(D)J Recombination Activity

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12 December 2020

Graft vs graft reaction resulting in the elimination of maternal cells in a SCID patient with maternofetal GVHd after an HLA identical bone marrow transplantation.

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12 December 2020

V(D)J recombination: a functional definition of the joining signals

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12 December 2020

Ku86-deficient mice exhibit severe combined immunodeficiency and defective processing of V(D)J recombination intermediates

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12 December 2020

Highly restricted human T cell repertoire in peripheral blood and tissue-infiltrating lymphocytes in Omenn's syndrome

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12 December 2020

RAG-1-deficient mice have no mature B and T lymphocytes

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12 December 2020

RAG1 mediates signal sequence recognition and recruitment of RAG2 in V(D)J recombination

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12 December 2020

RAG-1 and RAG-2, adjacent genes that synergistically activate V(D)J recombination

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12 December 2020

Complementation of V(D)J Recombination Deficiency in RAG-1−/− B Cells Reveals a Requirement for Novel Elements in the N-Terminus of RAG-1

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12 December 2020

Partial V(D)J recombination activity leads to Omenn syndrome

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12 December 2020

Cloning and characterization of the human recombination activating gene 1 (RAG1) and RAG2 promoter regions

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12 December 2020

Rch1, a protein that specifically interacts with the RAG-1 recombination-activating protein

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12 December 2020

V(D)J Recombination in Ku86-Deficient Mice: Distinct Effects on Coding, Signal, and Hybrid Joint Formation

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12 December 2020

Requirement for Stat4 in interleukin-12-mediated responses of natural killer and T cells

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12 December 2020

Familial Reticuloendotheliosis with Eosinophilia

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12 December 2020

The effect of antigen dose on CD4+ T helper cell phenotype development in a T cell receptor-alpha beta-transgenic model

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12 December 2020

A targeted DNA-PKcs-null mutation reveals DNA-PK-independent functions for KU in V(D)J recombination

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12 December 2020

DNA transposition by the RAG1 and RAG2 proteins: a possible source of oncogenic translocations

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12 December 2020

The pathology of Omenn's syndrome

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12 December 2020