This work had two purposes: (i) to determine in vivo whether liver phenylalanine hydroxylase (PAH) is regulated by its substrates phenylalanine and tetrahydrobiopterin (BH4) as studies with purified enzyme suggest and (ii) to investigate in vivo the relationship between PAH activity and BH4 turnover. We found there are two BH4 pools in hepatocytes, one that is metabolically available (free BH4)...

OBJECTIVES To describe the findings of cardiovascular magnetic resonance (CMR) imaging in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) and in consecutive patients with systemic sclerosis (SSc) without PAH. METHODS The study comprised nine consecutive patients who were admitted for right heart catheterization (RHC) under a suspicion of CTD...

Pulmonary artery hypertension (PAH) is an intractable disease of the small PAs in which multiple pathogenic factors are involved. Statins are known to mitigate endothelial injury and inhibit vascular remodeling and inflammation, all of which play crucial roles in the pathogenesis of PAH. We tested the hypothesis that nanoparticle (NP)-mediated delivery of pitavastatin into the lungs can be a no...

Pulmonary arterial hypertension (PAH) is a multi-factorial condition and the underlying pulmonary vascular disease is shaped by the combined action of genetic, epigenetic and immune-related factors. Whether and how gender, obesity and the metabolic syndrome modify PAH and associated right heart failure is under intense investigation. Estrogens may enhance the process of pulmonary angioprolifera...

In humans, liver phenylalanine hydroxylase (PAH) has an established catabolic function, and mutations in PAH cause phenylketonuria, a genetic disease characterized by neurological damage, if not treated. To obtain novel evolutionary insights and information on molecular mechanisms operating in phenylketonuria, we investigated PAH in the nematode Caenorhabditis elegans (cePAH), where the enzyme ...

background: one of the important etiologies for cryptogenic stroke is paradoxical embolization secondary to patent foramen ovale (pfo). foramen ovale can secondarily reopen due to pulmonary arterial hypertension (pah) which is common among the older age. pah is known as a frequent and life threatening complication of copd. the aim of this study was to determine the prevalence of pfo between cop...

This study explores the racial and ethnic differences in presentation, severity, and treatment of patients with pulmonary arterial hypertension (PAH) in a large multicenter registry. African American and Hispanic patients are more likely to present with associated PAH compared to non-Hispanic whites. Hispanic patients with PAH were less likely to be treated with PAH-specific medications compare...

Root-microbe interactions are considered to be the primary process of polyaromatic hydrocarbon (PAH) phytoremediation, since bacterial degradation has been shown to be the dominant pathway for environmental PAH dissipation. However, the precise mechanisms driving PAH rhizostimulation symbiosis remain largely unresolved. In this study, we assessed PAH degrading bacterial abundance in contaminate...

Suspended particles with the aerodynamic diameters not greater than 2.5 μm (PM2.5) and 1 μm (PM1, sub-fraction of PM2.5) were sampled at three sites: an urban background site, rural background site, and urban traffic site in southern Poland. In total, there were 240 samples taken within 02.08.2009-27.12.2010. Fifteen polycyclic aromatic hydrocarbons (PAH) were determined in each dust fraction. ...