American journal of respiratory and critical care medicine
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Am. J. Respir. Crit. Care Med. · Oct 2016
HIPPO-integrin Linked Kinase Crosstalk Controls Self-sustaining Proliferation and Survival in Pulmonary Hypertension.
Enhanced proliferation and impaired apoptosis of pulmonary arterial vascular smooth muscle cells (PAVSMCs) are key pathophysiologic components of pulmonary vascular remodeling in pulmonary arterial hypertension (PAH). ⋯ These data report inactivation of HIPPO/LATS1, self-supported via Yap-fibronectin-ILK1 signaling loop, as a novel mechanism of self-sustaining proliferation and apoptosis resistance of PAVSMCs in PAH and suggest a new potential target for therapeutic intervention.
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Am. J. Respir. Crit. Care Med. · Oct 2016
Comparative StudyNewborn Cystic Fibrosis Pigs Have a Blunted Early Response to an Inflammatory Stimulus.
Studies suggest that inappropriate responses to proinflammatory stimuli might contribute to inflammation in cystic fibrosis (CF) lungs. However, technical challenges have made it difficult to distinguish whether altered responses in CF airways are an intrinsic defect or a secondary effect of chronic disease in their tissue of origin. The CF pig model provides an opportunity to study the inflammatory responses of CF airways at birth, before the onset of infection and inflammation. ⋯ Transcriptome profiling suggests that acute inflammatory responses are dysregulated in the airways of newborn CF pigs.