(Q35147048)

6 August 2017

title

Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency (English)

1 reference

severe combined immunodeficiency

6 August 2017

main subject

1 reference

1 reference

Francesca Rucci

1

1 reference

6 August 2017

Pietro Luigi Poliani

2

1 reference

6 August 2017

Stefano Caraffi

3

1 reference

6 August 2017

Tiziana Paganini

4

1 reference

6 August 2017

Elena Fontana

5

1 reference

6 August 2017

Silvia Giliani

6

1 reference

6 August 2017

Frederick W Alt

7

1 reference

6 August 2017

Luigi Daniele Notarangelo

8

1 reference

6 August 2017

publication date

1 May 2011

1 reference

6 August 2017

published in

Frontiers in Immunology

1 reference

6 August 2017

volume

2

1 reference

6 August 2017

issue

15

1 reference

Lymphotoxin signals from positively selected thymocytes regulate the terminal differentiation of medullary thymic epithelial cells

6 August 2017

cites work

1 reference

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Expansion of immunoglobulin-secreting cells and defects in B cell tolerance in Rag-dependent immunodeficiency

13 July 2018

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Role of thymic cortex-specific self-peptides in positive selection of T cells

13 July 2018

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Hypomorphic Rag mutations can cause destructive midline granulomatous disease

13 July 2018

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Homozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndrome

13 July 2018

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Control of central self-tolerance induction by autoreactive CD4+ thymocytes

13 July 2018

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Lymphocyte proliferation in immune-mediated diseases

13 July 2018

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Early defects in human T-cell development severely affect distribution and maturation of thymic stromal cells: possible implications for the pathophysiology of Omenn syndrome

13 July 2018

1 reference

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Leaky severe combined immunodeficiency and aberrant DNA rearrangements due to a hypomorphic RAG1 mutation

13 July 2018

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A DNA-PKcs mutation in a radiosensitive T-B- SCID patient inhibits Artemis activation and nonhomologous end-joining.

13 July 2018

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Dendritic cells in the thymus contribute to T-regulatory cell induction

13 July 2018

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The impact of circulating dendritic cells on the development and differentiation of thymocytes

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

The earliest thymic progenitors for T cells possess myeloid lineage potential.

13 July 2018

1 reference

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13 July 2018

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Human primary immunodeficiency diseases

13 July 2018

1 reference

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Regulation of CD8+ T cell development by thymus-specific proteasomes

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

RANK signals from CD4(+)3(-) inducer cells regulate development of Aire-expressing epithelial cells in the thymic medulla

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

A hypomorphic R229Q Rag2 mouse mutant recapitulates human Omenn syndrome

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Selection of Foxp3+ regulatory T cells specific for self antigen expressed and presented by Aire+ medullary thymic epithelial cells

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Natural regulatory T cells: mechanisms of suppression

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Primary immunodeficiency syndromes associated with defective DNA double-strand break repair

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

RAG-dependent primary immunodeficiencies.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Clonal deletion of thymocytes by circulating dendritic cells homing to the thymus

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

A central role for central tolerance

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Journey through the thymus: stromal guides for T-cell development and selection

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Cernunnos, a novel nonhomologous end-joining factor, is mutated in human immunodeficiency with microcephaly

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Severe combined immunodeficiency and microcephaly in siblings with hypomorphic mutations in DNA ligase IV.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

A new type of radiosensitive T-B-NK+ severe combined immunodeficiency caused by a LIG4 mutation.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

A variant of SCID with specific immune responses and predominance of gamma delta T cells

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Central tolerance: learning self-control in the thymus

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Hassall's corpuscles instruct dendritic cells to induce CD4+CD25+ regulatory T cells in human thymus

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Linking signalling pathways, thymic stroma integrity and autoimmunity

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Promiscuous gene expression in thymic epithelial cells is regulated at multiple levels

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

AIRE deficiency in thymus of 2 patients with Omenn syndrome

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

An overview of three new disorders associated with genetic instability: LIG4 syndrome, RS-SCID and ATR-Seckel syndrome

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

The role of the non-homologous end-joining pathway in lymphocyte development

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Projection of an immunological self shadow within the thymus by the aire protein

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Lymphostromal interactions in thymic development and function

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Analysis of thymic stromal cell populations using flow cytometry

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Severe combined immunodeficiencies (SCID)

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Primary immunodeficiency diseases due to defects in lymphocytes

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

The RAG proteins and V(D)J recombination: complexes, ends, and transposition

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

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

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

RAG mutations in human B cell-negative SCID.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Thymic epithelial cells: the multi-tasking framework of the T cell "cradle".

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Thymus-specific serine protease regulates positive selection of a subset of CD4+ thymocytes

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Omenn syndrome is associated with mutations in DNA ligase IV.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

The cytokine RANKL produced by positively selected thymocytes fosters medullary thymic epithelial cells that express autoimmune regulator.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

The tumor necrosis factor family receptors RANK and CD40 cooperatively establish the thymic medullary microenvironment and self-tolerance.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

An immunodeficiency disease with RAG mutations and granulomas

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

A specific anti-Aire antibody reveals aire expression is restricted to medullary thymic epithelial cells and not expressed in periphery

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Characterization of the in vivo dynamics of medullary CD4+CD8- thymocyte development.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Medullary thymic epithelial cells expressing Aire represent a unique lineage derived from cells expressing claudin.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

A severe form of human combined immunodeficiency due to mutations in DNA ligase IV.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Developmental control point in induction of thymic cortex regulated by a subpopulation of prothymocytes.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

The lymphoid past of mouse plasmacytoid cells and thymic dendritic cells

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3150116

Phenotypic identification of the subgroups of murine T-cell receptor alphabeta+ CD4+ CD8- thymocytes and its implication in the late stage of thymocyte development

27 September 2018

1 reference

12 December 2020

Reduced central tolerance in Omenn syndrome leads to immature self-reactive oligoclonal T cells

1 reference

12 December 2020

Primary immunodeficiencies

1 reference

12 December 2020

Severe colitis in mice with aberrant thymic selection

1 reference

12 December 2020

Identification and Characterization of Thymic Epithelial Progenitor Cells

1 reference

12 December 2020

Autoantigen-Specific Interactions with CD4+ Thymocytes Control Mature Medullary Thymic Epithelial Cell Cellularity

1 reference

12 December 2020

Intrathymic restriction and peripheral expansion of the T-cell repertoire in Omenn syndrome

1 reference

12 December 2020

Production, selection, and maturation of thymocytes with high surface density of TCR

1 reference

12 December 2020

Steady-state dendritic cells continuously inactivate T cells that escape thymic negative selection

1 reference

12 December 2020

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

1 reference

12 December 2020

Abnormal development of thymic dendritic and epithelial cells in human X-linked severe combined immunodeficiency

1 reference

12 December 2020

Stepwise development of thymic microenvironments in vivo is regulated by thymocyte subsets

1 reference

12 December 2020

Defect of regulatory T cells in patients with Omenn syndrome

1 reference

12 December 2020

Identifiers

DOI

10.3389/FIMMU.2011.00015

1 reference

6 August 2017

1 reference

6 August 2017

1 reference