Human and animal models of V(D)J recombination deficiency (Q35538187)

8 August 2017

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title

Human and animal models of V(D)J recombination deficiency (English)

1 reference

8 August 2017

Jean-Pierre de Villartay

1

object named as

Jean-Pierre de Villartay

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Gwenaël Le Guyader

object named as

Gwenaël Le Guyader

5

1 reference

ORCID Public Data File 2021

author name string

Jean-Pierre de Villartay

1

1 reference

8 August 2017

Catherine Poinsignon

2

1 reference

8 August 2017

Regina de Chasseval

3

1 reference

8 August 2017

Dietke Buck

4

1 reference

8 August 2017

Isabelle Villey

6

1 reference

8 August 2017

1 October 2003

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Current Opinion in Immunology

8 August 2017

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8 August 2017

15

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8 August 2017

5

1 reference

8 August 2017

592-598

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The mechanism and regulation of chromosomal V(D)J recombination

8 August 2017

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

V(D)J recombination: RAG proteins, repair factors, and regulation

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

A recombinase diversified: new functions of the RAG proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Partners and pathwaysrepairing a double-strand break.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

V(D)J recombination and DNA repair: lessons from human immune deficiencies and other animal models

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

V(D)J recombination defects in lymphocytes due to RAG mutations: severe immunodeficiency with a spectrum of clinical presentations

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Primary immunodeficiency diseases: an experimental model for molecular medicine

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

The V(D)J recombination activating protein RAG2 consists of a six-bladed propeller and a PHD fingerlike domain, as revealed by sequence analysis

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Three-dimensional clustering of human RAG2 gene mutations in severe combined immune deficiency.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Identical mutations in RAG1 or RAG2 genes leading to defective V(D)J recombinase activity can cause either T-B-severe combined immune deficiency or Omenn syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Target-selected inactivation of the zebrafish rag1 gene

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

The PHD finger: implications for chromatin-mediated transcriptional regulation

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

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.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Dual role of RAG2 in V(D)J recombination: catalysis and regulation of ordered Ig gene assembly

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

The "dispensable" portion of RAG2 is necessary for efficient V-to-DJ rearrangement during B and T cell development

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Deletion of the RAG2 C terminus leads to impaired lymphoid development in mice

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

VDJ recombination: a transposase goes to work

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Effect of HIV integrase inhibitors on the RAG1/2 recombinase

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

The V(D)J recombinase efficiently cleaves and transposes signal joints

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

In vivo transposition mediated by V(D)J recombinase in human T lymphocytes

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

The C-terminal portion of RAG2 protects against transposition in vitro

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Regulation of RAG1/RAG2-mediated transposition by GTP and the C-terminal region of RAG2

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

The SCID mouse mutant: definition, characterization, and potential uses

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Ku86 is essential in human somatic cells

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Cytoprotective membrane-permeable peptides designed from the Bax-binding domain of Ku70.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Ku70 suppresses the apoptotic translocation of Bax to mitochondria.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Lack of detectable defect in DNA double-strand break repair and DNA-dependent protein kinase activity in radiosensitive human severe combined immunodeficiency fibroblasts

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

A new gene involved in DNA double-strand break repair and V(D)J recombination is located on human chromosome 10p

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Artemis, a novel DNA double-strand break repair/V(D)J recombination protein, is mutated in human severe combined immune deficiency.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

A founder mutation in Artemis, an SNM1-like protein, causes SCID in Athabascan-speaking Native Americans

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Radiosensitive SCID patients with Artemis gene mutations show a complete B-cell differentiation arrest at the pre-B-cell receptor checkpoint in bone marrow.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Novel Artemis gene mutations of radiosensitive severe combined immunodeficiency in Japanese families

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Metallo-beta-lactamase fold within nucleic acids processing enzymes: the beta-CASP family

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Hairpin opening and overhang processing by an Artemis/DNA-dependent protein kinase complex in nonhomologous end joining and V(D)J recombination

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Leaky Scid phenotype associated with defective V(D)J coding end processing in Artemis-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Targeted disruption of the gene encoding DNA ligase IV leads to lethality in embryonic mice

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

A critical role for DNA end-joining proteins in both lymphogenesis and neurogenesis

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Late embryonic lethality and impaired V(D)J recombination in mice lacking DNA ligase IV

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Identification of a defect in DNA ligase IV in a radiosensitive leukaemia patient

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Cellular and biochemical impact of a mutation in DNA ligase IV conferring clinical radiosensitivity

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

DNA ligase IV mutations identified in patients exhibiting developmental delay and immunodeficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

BCL2 oncogene translocation is mediated by a chi-like consensus

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Analysis of the V(D)J recombination efficiency at lymphoid chromosomal translocation breakpoints

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

DNA double strand break repair and chromosomal translocation: lessons from animal models

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Cancer-susceptibility genes. Gatekeepers and caretakers

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Evidence for replicative repair of DNA double-strand breaks leading to oncogenic translocation and gene amplification

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Unrepaired DNA breaks in p53-deficient cells lead to oncogenic gene amplification subsequent to translocations.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Impaired nonhomologous end-joining provokes soft tissue sarcomas harboring chromosomal translocations, amplifications, and deletions

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Defective DNA repair and increased genomic instability in Artemis-deficient murine cells

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Partial T and B lymphocyte immunodeficiency and predisposition to lymphoma in patients with hypomorphic mutations in Artemis

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Nonhomologous end joining and V(D)J recombination require an additional factor

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

A systematic, genome-wide, phenotype-driven mutagenesis programme for gene function studies in the mouse

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Genome-wide, large-scale production of mutant mice by ENU mutagenesis.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Genome-wide ENU mutagenesis to reveal immune regulators

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

The art and design of genetic screens: zebrafish

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

Myc-induced T cell leukemia in transgenic zebrafish.

1 reference

https://api.crossref.org/works/10.1016%2FS0952-7915%2803%2900101-8

7 January 2021

10.1016/S0952-7915(03)00101-8

Identifiers

DOI

1 reference

8 August 2017

1 reference