Chronic NOS inhibition prevents adverse lung remodeling and pulmonary arterial hypertension in caveolin-1 knockout mice

C Wunderlich, A Schmeisser, C Heerwagen… - Pulmonary …, 2008 - Elsevier
Recently generated caveolin-1 deficient mice (cav-1 ko) suffer from severe lung fibrosis with
marked pulmonary hypertension and arterial hypoxemia and may therefore serve as an
useful animal model of this devastating human disorder. Accumulating evidence strongly
supports the negative regulatory influence of caveolin-1 on endothelial nitric oxide synthase
resulting in a constitutive hyperactivation of the nitric oxide (NO) pathway in cav-1 ko. We
therefore hypothesized that a disturbed NO signaling is implicated in the evolution of the …