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Article

A Defect in Thymic Tolerance Causes T Cell-Mediated Autoimmunity in a Murine Model of COPA Syndrome.

Journal of immunology (Baltimore, Md. : 1950) - 1 May 2020

Deng Zimu, Law Christopher S, Ho Frances O, Wang Kristin M, Jones Kirk D, Shin Jeoung-Sook, Shum Anthony K

Abstract excerpt

COPA syndrome is a recently described Mendelian autoimmune disorder caused by missense mutations in the coatomer protein complex subunit α (COPA) gene. Patients with COPA syndrome develop arthritis and lung disease that presents as pulmonary hemorrhage or interstitial lung disease (ILD). Immunosuppressive medications can stabilize the disease, but many patients develop progressive pulmonary fibrosis, which...

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Identifiers

PMID: 32198142
DOI: 10.4049/jimmunol.2000028
PMCID: PMC7324259

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A Defect in Thymic Tolerance Causes T Cell-Mediated Autoimmunity in a Murine Model of COPA Syndrome.PMID 32198142Read ↗

A defect in thymic tolerance causes T cell-mediated autoimmunity in a murine model of COPA syndromeSemantic proximity Read ↗

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