(Q37262455)

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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

scientific article published on 04 May 2009

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Europe PubMed Central

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27 January 2020

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

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27 January 2020

pathophysiology

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Pietro Luigi Poliani

1

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27 January 2020

Fabio Facchetti

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27 January 2020

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27 January 2020

Luigi D Notarangelo

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Luigi D Notarangelo

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27 January 2020

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Maria Ravanini

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27 January 2020

Andrew Richard Gennery

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27 January 2020

Chaim M Roifman

6

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27 January 2020

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4 May 2009

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27 January 2020

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Europe PubMed Central

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27 January 2020

114

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27 January 2020

1

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27 January 2020

105-108

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27 January 2020

Omenn syndrome: inflammation in leaky severe combined immunodeficiency

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

Human primary immunodeficiency diseases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

A hypomorphic R229Q Rag2 mouse mutant recapitulates human Omenn syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

AIRE deficiency in thymus of 2 patients with Omenn syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

Normal thymic architecture and negative selection are associated with Aire expression, the gene defective in the autoimmune-polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

Interdependence of cortical thymic epithelial cell differentiation and T-lineage commitment

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

6 September 2017

An interleukin-2 receptor gamma chain mutation with normal thymus morphology

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

3 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

3 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

3 September 2018

Mutations in the RNA component of RNase mitochondrial RNA processing might cause Omenn syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

3 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2710940

3 September 2018

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/19414857

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/19414857

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Claudins provide a breath of fresh Aire

1 reference

https://pubmed.ncbi.nlm.nih.gov/19414857

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/19414857

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/19414857

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1182/BLOOD-2009-03-211029

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19414857%20AND%20SRC:MED&resulttype=core&format=json

27 January 2020

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19414857%20AND%20SRC:MED&resulttype=core&format=json

27 January 2020

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19414857%20AND%20SRC:MED&resulttype=core&format=json

27 January 2020

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