Article

A defect in thymic tolerance causes T cell-mediated autoimmunity in a murine model of COPA syndrome

2020-01-22

Abstract excerpt

COPA syndrome is a recently described Mendelian autoimmune disorder caused by missense mutations in the Coatomer protein complex subunit alpha (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 that requ...

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Literature Corpus work: 9df992f0-ee9b-5097-a364-11ac3f16cbc2
DOI: 10.1101/2020.01.21.914440

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