Pulmonary arterial hypertension (PAH) is a serious and often fatal disease. It is a panvasculopathy of the pulmonary microcirculation characterized by vasoconstriction and arterial obstruction due to vascular proliferation and remodeling and ultimately right ventricular failure. Mitochondrial dysfunction is a universal finding in pulmonary vascular cells of patients with PAH, and is mechanistic...

Pulmonary arterial hypertension (PAH) is an intractable disease of the small pulmonary artery that involves multiple inflammatory factors. We hypothesized that a redox-sensitive transcription factor, nuclear factor B (NFB), which regulates important inflammatory cytokines, plays a pivotal role in PAH. We investigated the activity of NFB in explanted lungs from patients with PAH and in a rat mod...

This study employed an in vitro version of the lacZ transgenic rodent mutation assay to assess the mutagenicity of nonpolar neutral and semipolar aromatic soil fractions from 10 PAH-contaminated sites, and evaluated the assumption of dose additivity that is routinely employed to calculate the risk posed by PAH mixtures. Significant mutagenic activity was detected in all nonpolar neutral fractio...

215 interleukin 6, the expression of which is also modulated by BMPR2.5 The currently enrolling clinical trials in PAH with molecules such as FK506, rituximab, and anakinra selectively targeting the immune system are promising. In the study by Jasiewicz et al.,3 10 patients were incident cases who received PAH-targeted therapy at entry to the study. It would be interesting to see whether the le...

OBJECTIVE Germline mutations in the bone morphogenetic protein receptor type-2 (BMPR2) gene are considered to be a major risk factor for pulmonary arterial hypertension (PAH). BMPR2 mutations have been reported in 10%-20% of idiopathic PAH and in 80% of familial PAH cases. The aim of this study was to evaluate the frequency of mutations in the serine/threonine kinase domain of the BMPR2 gene in...

Pulmonary hypertension (PH) is found in a vast array of diseases, with a minority representing pulmonary arterial hypertension (PAH). Idiopathic PAH or PAH in association with other disorders has been associated with poor survival, poor exercise tolerance, progressive symptoms of dyspnea, and decreased quality of life. Left untreated, patients with PAH typically have a progressive decline in fu...

Alfalfa, tall fescue, ryegrass, and celery, some of which were inoculated with the AM fungus Glomus intraradices, were compared for their contributions to phenanthrene (PHE), pyrene (PYR), and dibenzo(a,h)anthracene (DBA) dissipation in spiked soil. A pot experiment was conducted in which PAHs extracted from soil and plant, quantity of PAH degraders, and plant biomass were evaluated. The result...

Pulmonary arterial hypertension (PAH) is a devastating disease in which remodeling of the small pulmonary arteries leads to a progressive increase in pulmonary vascular resistance and right-sided heart failure. Over the past decade, new treatments for PAH, such as the use of ERAs, PDE-5 inhibitors and prostacyclin analogs, have brought about dramatic improvements in clinical outcomes. Epoproste...

Depending on their origins, polycyclic aromatic hydrocarbons (PAH) are characterized by different chemical properties. Petrogenic PAH (e.g. from fossil fuels) and pyrolytic PAH (e.g. those produced by incineration processes) are therefore expected to affect organisms differently. The impact of trophic exposure to these PAH was investigated on swimming and metabolic performance of zebrafish Dani...

Mutation analysis in a patient with mild hyperphenylalaninaemia showed three distinct base substitutions in exon 12 of the phenylalanine hydroxylase (PAH) gene. All three mutations, R413P, Y414C, and D415N, have previously been described as being independently associated with PAH deficiency. Family studies and independent analysis of the PAH alleles of the patient showed cosegregation of the R4...