Impaired innate host defense causes susceptibility to respiratory virus infections in cystic fibrosis.

摘要: Viral infection is the primary cause of respiratory morbidity in cystic fibrosis (CF) infants. Here, we identify that host factors allow increased virus replication and cytokine production, providing a mechanism for understanding severity disease CF. Increased due to lack nitric oxide synthase 2 (NOS2) 2', 5' oligoadenylate synthetase (OAS) 1 induction response or IFNgamma. This can be attributed impairment activation signal transducer activator transcription (STAT)1, fundamental component antiviral defense. NO donor NOS2 overexpression provides protection from CF, suggesting sufficient defense human airway one strategy therapy CF children.