Lung fibrosis is associated with inflammation, apoptosis and oxidative damage. The transcription factor nuclear factor erythroid 2‑related factor‑2 (Nrf2) prevents damage to cells from oxidative stress by regulating the expression of antioxidant proteins. Sulforaphane (SFN), an Nrf2 activator, additionally regulates excessive oxidative stress by promoting the expression of endogenous antioxidan...

The severity of cystic fibrosis (CF) pulmonary disease is not directly related to CFTR genotype but depends upon several parameters, including neutrophil-dominated inflammation. Identification of agents modulating inflammation constitutes a relevant goal. Myeloperoxidase (MPO) is involved in both microbicidal and proinflammatory neutrophil activities. The aim of this study was to evaluate wheth...

In vivo lung micro-CT is increasingly embraced in pulmonary research, because it provides longitudinal information on dynamic disease processes in a field where ex vivo assessment of experimental disease models is still the gold standard. To optimize the quantitative monitoring of progression and therapy of lung diseases, we evaluated longitudinal changes in four different micro-CT-derived biom...

Abstract Background Systemic sclerosis (SSc) is characterized by vasculopathy, inflammation and fibrosis, carries one of the worst prognosis in patients who also develop pulmonary arterial hypertension (PAH). Although PAH a known prognosticator, SSc-PAH demonstrate disproportionately high morbidity mortality, presumably due to cardiac involvement. Purpose To determine pathophysiology involvemen...

Pulmonary fibrosis is a progressive and lethal lung disease characterized by accumulation of extracellular matrix and loss of pulmonary function. No cure exists for this pathologic condition, and current treatments often fail to slow its progression or relieve its symptoms. Relaxin was previously shown to induce a matrix-degrading phenotype in human lung fibroblasts in vitro and to inhibit pulm...

The mechanisms underlying the pathogenesis of idiopathic pulmonary fibrosis (IPF) involve multiple pathways, such as inflammation, epithelial mesenchymal transition, coagulation, oxidative stress, and developmental processes. The small GTPase, RhoA, and its target protein, Rho-kinase (ROCK), may interact with other signaling pathways known to contribute to pulmonary fibrosis. This study aimed t...

Pulmonary inflammation, abnormalities in type II cell and macrophage morphology, and pulmonary fibrosis are features of Hermansky-Pudlak Syndrome (HPS), a recessive disorder associated with intracellular trafficking defects. We have previously reported that "Pearl" (HPS2) and "Pale Ear" (HPS1) mouse models have pulmonary inflammatory dysregulation and constitutive alveolar macrophage (AM) activ...

BACKGROUND In idiopathic pulmonary fibrosis loss of alveolar epithelium induces inflammation of the pulmonary tissue followed by accumulation of pathogenic myofibroblasts leading eventually to respiratory failures. In animal models inflammatory and resident cells have been demonstrated to contribute to pulmonary fibrosis. Regenerative potential of pulmonary and extra-pulmonary stem and progenit...

pulmonary fibrosis is a progressive disease of the lungs, which leads to death in human. it has been suggested that transforming growth factor beta 1 (tgf-β1) together with oxidative stress play a central role in the pathogenesis of the ailment. the objective of this study was to evaluate the possible curative effects of black radish, raphanus sativus l . var niger (rsn) on bleomycine (blm) -in...

Patients with cystic fibrosis (CF) suffer from chronic infections and severe inflammation, which lead to progressive pulmonary disease [1]. Chronic suppurative lung disease, pancreatic insufficiency and anorexia are interrelated complications that have negative impacts on food intake and may affect the energy balance, contributing to the malnutrition [2]. Nutritional status is a prognostic mark...