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Abstract

References (150)

Projection of an Immunological Self Shadow Within the Thymus by the Aire Protein

2002 • 2,371 citations

A Chromatin Landmark and Transcription Initiation at Most Promoters in Human Cells

2007 • 1,882 citations

Thymic selection of CD4+CD25+ regulatory T cells induced by an agonist self-peptide

2001 • 1,659 citations

Positional cloning of the APECED gene

1997 • 1,385 citations

An autoimmune disease, APECED, caused by mutations in a novel gene featuring two PHD-type zinc-finger domains

1997 • 1,155 citations

Integrating mRNA Processing with Transcription

2002 • 1,054 citations

The insulin gene is transcribed in the human thymus and transcription levels correlate with allelic variation at the INS VNTR-IDDM2 susceptibility locus for type 1 diabetes

1997 • 931 citations

ING2 PHD domain links histone H3 lysine 4 methylation to active gene repression

2006 • 916 citations

Origin of regulatory T cells with known specificity for antigen

2002 • 854 citations

Insulin expression in human thymus is modulated by INS VNTR alleles at the IDDM2 locus

1997 • 806 citations

A Topoisomerase IIß-Mediated dsDNA Break Required for Regulated Transcription

2006 • 797 citations

Aire regulates negative selection of organ-specific T cells

2003 • 789 citations

RNA polymerase is poised for activation across the genome

2007 • 744 citations

V(D)J Recombination: RAG Proteins, Repair Factors, and Regulation

2002 • 734 citations

A CENTRAL ROLE FOR CENTRAL TOLERANCE

2006 • 730 citations

Cellular and genetic mechanisms of self tolerance and autoimmunity

2005 • 671 citations

The Cellular Mechanism of Aire Control of T Cell Tolerance

2005 • 622 citations

The enemy within: keeping self-reactive T cells at bay in the periphery

2002 • 586 citations

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

2007 • 562 citations

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

2005 • 540 citations

Targeting histone deacetylase complexes via KRAB-zinc finger proteins: the PHD and bromodomains of KAP-1 form a cooperative unit that recruits a novel isoform of the Mi-2α subunit of NuRD

2001 • 504 citations

Central Tolerance to Tissue-specific Antigens Mediated by Direct and Indirect Antigen Presentation

2004 • 474 citations

Deletional Tolerance Mediated by Extrathymic Aire-Expressing Cells

2008 • 468 citations

The Tumor Necrosis Factor Family Receptors RANK and CD40 Cooperatively Establish the Thymic Medullary Microenvironment and Self-Tolerance

2008 • 464 citations

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

2007 • 463 citations

Aire deficient mice develop multiple features of APECED phenotype and show altered immune response

2002 • 463 citations

Recognition of unmethylated histone H3 lysine 4 links BHC80 to LSD1-mediated gene repression

2007 • 418 citations

Anti-Interferon Autoantibodies in Autoimmune Polyendocrinopathy Syndrome Type 1

2006 • 418 citations

The Cytokine RANKL Produced by Positively Selected Thymocytes Fosters Medullary Thymic Epithelial Cells that Express Autoimmune Regulator

2008 • 405 citations

The PHD finger, a nuclear protein-interaction domain

2005 • 403 citations

An autosomal locus causing autoimmune disease: autoimmune polyglandular disease type I assigned to chromosome 21

1994 • 379 citations

Proliferative arrest and rapid turnover of thymic epithelial cells expressing Aire

2007 • 367 citations

Thymic Medullary Epithelial Cell Differentiation, Thymocyte Emigration, and the Control of Autoimmunity Require Lympho–Epithelial Cross Talk via LTβR

2003 • 361 citations

Peripheral antigen display by lymph node stroma promotes T cell tolerance to intestinal self

2006 • 346 citations

Cutting Edge: The Prevalence of Regulatory T Cells in Lymphoid Tissue Is Correlated with Viral Load in HIV-Infected Patients

2005 • 337 citations

Yng1 PHD Finger Binding to H3 Trimethylated at K4 Promotes NuA3 HAT Activity at K14 of H3 and Transcription at a Subset of Targeted ORFs

2006 • 327 citations

Nutrient regulates Tor1 nuclear localization and association with rDNA promoter

2006 • 303 citations

Autoimmune Regulator Is Expressed in the Cells Regulating Immune Tolerance in Thymus Medulla

1999 • 293 citations

Gene Dosage–limiting Role ofAirein Thymic Expression, Clonal Deletion, and Organ-specific Autoimmunity

2004 • 291 citations

Does T-cell tolerance require a dedicated antigen-presenting cell?

1989 • 288 citations

Development of Autoimmunity against Transcriptionally Unrepressed Target Antigen in the Thymus of Aire-Deficient Mice

2005 • 287 citations

Dynamics of Thymocyte-Stromal Cell Interactions Visualized by Two-Photon Microscopy

2002 • 278 citations

Crosstalk in the mouse thymus

1994 • 275 citations

Dependence of Self-Tolerance on TRAF6-Directed Development of Thymic Stroma

2005 • 272 citations

Spontaneous autoimmunity prevented by thymic expression of a single self-antigen

2006 • 271 citations

Normal Thymic Architecture and Negative Selection Are Associated with Aire Expression, the Gene Defective in the Autoimmune-Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED)

2000 • 261 citations

Insulin Expression Levels in the Thymus Modulate Insulin-Specific Autoreactive T-Cell Tolerance

2002 • 260 citations

The Autoimmune Regulator Protein Has Transcriptional Transactivating Properties and Interacts with the Common Coactivator CREB-binding Protein

2000 • 239 citations

The LxxLL motif: a multifunctional binding sequence in transcriptional regulation

2004 • 234 citations

Modifier loci condition autoimmunity provoked by Aire deficiency

2005 • 233 citations

The autoimmune regulator PHD finger binds to non‐methylated histone H3K4 to activate gene expression

2008 • 226 citations

The multi-tasking P-TEFb complex

2008 • 225 citations

Negative Selection in the Thymus Includes Semimature T Cells

1997 • 224 citations

The SAND domain structure defines a novel DNA-binding fold in transcriptional regulation.

2001 • 222 citations

Primary immunodeficiency diseases: An update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005

2006 • 220 citations

A Defect of Regulatory T Cells in Patients with Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy

2007 • 217 citations

Diverse roles of androgen receptor (AR) domains in AR-mediated signaling

2008 • 212 citations

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

2007 • 207 citations

Targeted Complementation of MHC Class II Deficiency by Intrathymic Delivery of Recombinant Adenoviruses

1997 • 204 citations

Structural Mechanism of Smad4 Recognition by the Nuclear Oncoprotein Ski

2002 • 204 citations

Back to Central Tolerance

2004 • 202 citations

A Novel Mutation of the Autoimmune Regulator Gene in an Italian Kindred with Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy, Acting in a Dominant Fashion and Strongly Cosegregating with Hypothyroid Autoimmune Thyroiditis

2001 • 199 citations

Promiscuous gene expression and central T-cell tolerance: more than meets the eye

2002 • 198 citations

AIRE Mutations and Human Leukocyte Antigen Genotypes as Determinants of the Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy Phenotype

2002 • 195 citations

Lymphotoxin pathway directs thymic Aire expression

2003 • 193 citations

Promiscuous gene expression patterns in single medullary thymic epithelial cells argue for a stochastic mechanism

2008 • 191 citations

A decade of AIRE

2007 • 188 citations

Thymic emigration: conveyor belts or lucky dips?

1995 • 186 citations

RNA and protein expression of the murine autoimmune regulator gene (Aire) in normal, RelB-deficient and in NOD mouse

2000 • 185 citations

An IRF8-binding promoter variant and AIRE control CHRNA1 promiscuous expression in thymus

2007 • 181 citations

Aire employs a histone-binding module to mediate immunological tolerance, linking chromatin regulation with organ-specific autoimmunity

2008 • 167 citations

Mechanisms of an autoimmunity syndrome in mice caused by a dominant mutation in Aire

2008 • 164 citations

Localization of the APECED protein in distinct nuclear structures

1999 • 154 citations

AIRE Recruits P-TEFb for Transcriptional Elongation of Target Genes in Medullary Thymic Epithelial Cells

2007 • 151 citations

AIRE Functions As an E3 Ubiquitin Ligase

2004 • 145 citations

Promiscuous gene expression and the developmental dynamics of medullary thymic epithelial cells

2007 • 142 citations

Expression of AIRE gene in peripheral monocyte/dendritic cell lineage

2002 • 130 citations

Subcellular Localization of the Autoimmune Regulator Protein

2001 • 127 citations

Chromosomal clustering of genes controlled by the aire transcription factor

2005 • 126 citations

Nucleosome Binding by the Bromodomain and PHD Finger of the Transcriptional Cofactor p300

2004 • 125 citations

Interferon autoantibodies associated with AIRE deficiency decrease the expression of IFN-stimulated genes

2008 • 123 citations

Ectopic expression of peripheral-tissue antigens in the thymic epithelium: Probabilistic, monoallelic, misinitiated

2008 • 120 citations

ULTRAPETALA1encodes a SAND domain putative transcriptional regulator that controls shoot and floral meristem activity inArabidopsis

2005 • 120 citations

How defects in central tolerance impinge on a deficiency in regulatory T cells

2005 • 117 citations

Alteration of intra-pancreatic target-organ specificity by abrogation of Aire in NOD mice

2006 • 117 citations

Medullary Thymic Epithelium: A Mosaic of Epithelial “Self”?

1998 • 114 citations

The genetic background of autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy and its autoimmune disease components

2002 • 114 citations

AIRE encodes a nuclear protein co-localizing with cytoskeletal filaments: altered sub-cellular distribution of mutants lacking the PHD zinc fingers

1999 • 113 citations

Organization of thymic medullary epithelial heterogeneity: implications for mechanisms of epithelial differentiation

2002 • 111 citations

ACF1 improves the effectiveness of nucleosome mobilization by ISWI through PHD–histone contacts

2004 • 111 citations

APECED-causing mutations in AIRE reveal the functional domains of the protein

2004 • 109 citations

The transition from transcriptional initiation to elongation

2008 • 109 citations

Polycomblike PHD Fingers Mediate Conserved Interaction with Enhancer of Zeste Protein

2001 • 108 citations

Defective Neural Tube Closure and Anteroposterior Patterning in Mice Lacking the LIM Protein LMO4 or Its Interacting Partner Deaf-1

2004 • 108 citations

The Lymphotoxin Pathway Regulates Aire-Independent Expression of Ectopic Genes and Chemokines in Thymic Stromal Cells

2008 • 107 citations

A Specific Anti-Aire Antibody Reveals Aire Expression Is Restricted to Medullary Thymic Epithelial Cells and Not Expressed in Periphery

2008 • 105 citations

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED): a model disease to study molecular aspects of endocrine autoimmunity

2004 • 103 citations

Autoimmune Polyglandular Syndrome Type 1

1998 • 103 citations

CHRONIC IDIOPATHIC HYPOPARATHYROIDISM WITH SUPERIMPOSED ADDISON'S DISEASE IN A CHILD

1946 • 101 citations

APECED: a monogenic autoimmune disease providing new clues to self-tolerance

1998 • 99 citations

The insulin gene in diabetes

2002 • 98 citations

Subcellular Location and Expression Pattern of Autoimmune Regulator (Aire), the Mouse Orthologue for Human Gene Defective in Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)

2001 • 97 citations

AIRE's CARD Revealed, a New Structure for Central Tolerance Provokes Transcriptional Plasticity

2007 • 95 citations

Lymphotoxin Pathway and Aire Influences on Thymic Medullary Epithelial Cells Are Unconnected

2007 • 95 citations

Loss of Aire-dependent thymic expression of a peripheral tissue antigen renders it a target of autoimmunity

2007 • 95 citations

NMR Structure of the First PHD Finger of Autoimmune Regulator Protein (AIRE1)

2005 • 89 citations

Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy Syndrome: Time to Review Diagnostic Criteria?

2003 • 86 citations

Sequential phases in the development of Aire‐expressing medullary thymic epithelial cells involve distinct cellular input

2008 • 85 citations

Role of the Autoimmune Regulator (AIRE) gene in alopecia areata: Strong association of a potentially functional AIRE polymorphism with alopecia universalis

2002 • 85 citations

B cells are required for Aire-deficient mice to develop multi-organ autoinflammation: A therapeutic approach for APECED patients

2008 • 84 citations

Expression of Aire and the Early Wave of Apoptosis in Spermatogenesis

2008 • 82 citations

Increased antigen presenting cell‐mediated T cell activation in mice and patients without the autoimmune regulator

2006 • 81 citations

Danger-free autoimmune disease in Aire-deficient mice

2007 • 81 citations

Effector Mechanisms of the Autoimmune Syndrome in the Murine Model of Autoimmune Polyglandular Syndrome Type 1

2008 • 80 citations

Pygopus Residues Required for its Binding to Legless Are Critical for Transcription and Development

2004 • 79 citations

Evidence for a single-niche model of positive selection.

1994 • 76 citations

Highly variable expression of tissue‐restricted self‐antigens in human thymus: Implications for self‐tolerance and autoimmunity

2007 • 72 citations

Subcellular Expression of Autoimmune Regulator Is Organized in a Spatiotemporal Manner

2004 • 71 citations

DNA-PK contributes to the phosphorylation of AIRE: Importance in transcriptional activity

2007 • 71 citations

Association Analysis of the Cytotoxic T Lymphocyte Antigen-4 (CTLA-4) and Autoimmune Regulator-1 (AIRE-1) Genes in Sporadic Autoimmune Addison's Disease

2000 • 68 citations

Isolation and Characterization of the MouseAireGene

1999 • 68 citations

Contrasting models of promiscuous gene expression by thymic epithelium

2005 • 67 citations

Cooperative activation of transcription by autoimmune regulator AIRE and CBP

2005 • 66 citations

Incomplete block of B cell development and immunoglobulin production in mice carrying the ? MT mutation on the BALB/c background

2002 • 64 citations

Cloning ofAire,the Mouse Homologue of the Autoimmune Regulator (AIRE) Gene Responsible for Autoimmune Polyglandular Syndrome Type 1 (APS1)

1999 • 60 citations

Screening for an AIRE‐1 mutation in patients with Addison's disease, type 1 diabetes, Graves' disease and Hashimoto's thyroiditis as well as in APECED syndrome

2001 • 59 citations

Aire deficient mice do not develop the same profile of tissue-specific autoantibodies as APECED patients

2006 • 58 citations

Evolutionarily conserved and divergent regions of the Autoimmune Regulator (Aire) gene: a comparative analysis

2008 • 58 citations

Transcriptional impact of Aire varies with cell type

2008 • 57 citations

Autoimmune regulator induced changes in the gene expression profile of human monocyte-dendritic cell-lineage

2004 • 54 citations

The variable immunological self: Genetic variation and nongenetic noise in Aire-regulated transcription

2008 • 54 citations

Solution structure and NMR characterization of the binding to methylated histone tails of the plant homeodomain finger of the tumour suppressor ING4

2006 • 51 citations

Lymphotoxin Pathway-Directed, Autoimmune Regulator-Independent Central Tolerance to Arthritogenic Collagen

2006 • 51 citations

Mutational analysis of the autoimmune regulator (AIRE) gene in sporadic autoimmune Addison's disease can reveal patients with unidentified autoimmune polyendocrine syndrome type I

2002 • 45 citations

Association analysis of the AIRE and insulin genes in Finnish type 1 diabetic patients

2006 • 44 citations

Crystal Structure and Nuclear Magnetic Resonance Analyses of the SAND Domain from Glucocorticoid Modulatory Element Binding Protein-1 Reveals Deoxyribonucleic Acid and Zinc Binding Regions

2003 • 44 citations

Critical immunological pathways are downregulated in APECED patient dendritic cells

2008 • 44 citations

Distinct Clinical Phenotype and Immunoreactivity in Japanese Siblings with Autoimmune Polyglandular Syndrome Type 1 (APS-1) Associated with Compound Heterozygous Novel AIRE Gene Mutations

2002 • 41 citations

Expression profiling of autoimmune regulator AIRE mRNA in a comprehensive set of human normal and neoplastic tissues

2006 • 37 citations

Role of PHD fingers and COOH-terminal 30 amino acids in AIRE transactivation activity

2007 • 36 citations

Detection of a complete autoimmune regulator gene deletion and two additional novel mutations in a cohort of patients with atypical phenotypic variants of autoimmune polyglandular syndrome type 1

2008 • 35 citations

AIRE recruits multiple transcriptional components to specific genomic regions through tethering to nuclear matrix

2005 • 35 citations

SP100B, a repressor of gene expression preferentially binds to DNA with unmethylated CpGs

2006 • 34 citations

Investigation of the p.Ser278Arg polymorphism of the autoimmune regulator (AIRE) gene in alopecia areata

2006 • 32 citations

Immunological Findings in Patients with Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) and their Family Members: Are Heterozygotes Subclinically Affected?

2002 • 31 citations

A consensus DNA recognition motif for two KDWK transcription factors identifies flexible-length, CpG-methylation sensitive cognate binding sites in the majority of human promoters

2001 • 25 citations

-Cell Autoantibodies, Human Leukocyte Antigen II Alleles, and Type 1 Diabetes in Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy

2000 • 24 citations

Aire downregulates multiple molecules that have contradicting immune-enhancing and immune-suppressive functions

2004 • 24 citations

Innate and adaptive immunity in patients with autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy

2008 • 12 citations

Aire Deficiency Causes Increased Susceptibility to Streptozotocin‐induced Murine Type 1 Diabetes

2008 • 9 citations

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Aire (2009) – Annual Review of Immunology | Metascience Observatory Explorer