ArticleJournal of immunology (Baltimore, Md. : 1950)2012
Defective autoimmune regulator-dependent central tolerance to myelin protein zero is linked to autoimmune peripheral neuropathy.
Article in Journal of immunology (Baltimore, Md. : 1950), 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
35 citing papers in PubMed, 53 citations in OpenAlex.
- Age-associated Autoimmunity Driven by T cell Immunosenescence.bioRxiv : the preprint server for biology · 2026Article
- NETs mediate neuropathic pain in PSS-PN by participating in inflammatory injury to the vasa nervorum.Clinical rheumatology · 2026Article
- Microbiota and mycobiota in chronic inflammatory demyelinating polyneuropathy: clinical and pathophysiological correlations.Frontiers in molecular neuroscience · 2026Article
- Genetic and metabolic inflammation signatures in chronic inflammatory demyelinating polyneuropathy: the role ofFrontiers in molecular neuroscience · 2025Article
- Animal models of immune-mediated demyelinating polyneuropathies.Autoimmunity · 2024Review
- A pathologically expanded, clonal lineage of IL-21-producing CD4+ T cells drives inflammatory neuropathy.The Journal of clinical investigation · 2024Article
- A pathologically expanded, clonal lineage of IL-21 producing CD4+ T cells drives Inflammatory neuropathy.bioRxiv : the preprint server for biology · 2024Article
- Pathogenic TNF-α drives peripheral nerve inflammation in an Aire-deficient model of autoimmunity.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
- The NOD Mouse Beyond Autoimmune Diabetes.Frontiers in immunology · 2022Review
- The immune response and aging in chronic inflammatory demyelinating polyradiculoneuropathy.Journal of neuroinflammation · 2021Review
- Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy.Frontiers in pediatrics · 2021Review
- Deciphering immune mechanisms in chronic inflammatory demyelinating polyneuropathies.JCI insight · 2020Review
- The presentation of neuroendocrine self-peptides in the thymus: an essential event for individual life and vertebrate survival.Annals of the New York Academy of Sciences · 2019Review
- Lessons from primary immunodeficiencies: Autoimmune regulator and autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy.Immunological reviews · 2019Review
- Deciphering Molecular and Phenotypic Changes Associated with Early Autoimmune Disease in the Aire-Deficient Mouse Model of Sjögren's Syndrome.International journal of molecular sciences · 2018Article
- Schwann cell-derived periostin promotes autoimmune peripheral polyneuropathy via macrophage recruitment.The Journal of clinical investigation · 2018Article
- IL-10 Paradoxically Promotes Autoimmune Neuropathy through S1PR1-Dependent CD4Journal of immunology (Baltimore, Md. : 1950) · 2018Article
- Twenty Years of AIRE.Frontiers in immunology · 2018Review
- Insights from the sequence similarity of Zika virus proteins with the Human nerve proteins.Bioinformation · 2018Article
- Peculiarities of autoimmune polyglandular syndromes in children and adolescents.Acta bio-medica : Atenei Parmensis · 2017Review
Corrections and comments
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Authors and funding
13 authors at 5 institutions in 3 countries.
Funding
Abstract
Chronic inflammatory demyelinating polyneuropathy is a debilitating autoimmune disease characterized by peripheral nerve demyelination and dysfunction. How the autoimmune response is initiated, identity of provoking Ags, and pathogenic effector mechanisms are not well defined. The autoimmune regulator (Aire) plays a critical role in central tolerance by promoting thymic expression of self-Ags and deletion of self-reactive T cells. In this study, we used mice with hypomorphic Aire function and two patients with Aire mutations to define how Aire deficiency results in spontaneous autoimmune peripheral neuropathy. Autoimmunity against peripheral nerves in both mice and humans targets myelin protein zero, an Ag for which expression is Aire-regulated in the thymus. Consistent with a defect in thymic tolerance, CD4(+) T cells are sufficient to transfer disease in mice and produce IFN-γ in infiltrated peripheral nerves. Our findings suggest that defective Aire-mediated central tolerance to myelin protein zero initiates an autoimmune Th1 effector response toward peripheral nerves.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.