COPA mutations impair ER-Golgi transport and cause hereditary autoimmune-mediated lung disease and arthritis

nthony Shum, Jordan Orange, James Lupski and colleagues show that mutations in the coatamer subunit gene COPA cause an autoimmune syndrome characterized by inflammatory arthritis and interstitial lung disease. They further show that the mutations disrupt ER-Golgi transport, leading to ER stress and altered T cell function.