Search results for "LSID"

Landslide analysis in the Iato river basin (north-western Sicily, Italy)

2006

Chemical model systems for cellular nitros(yl)ation reactions.

2009

S-nitros(yl)ation belongs to the redox-based posttranslational modifications of proteins but the underlying chemistry is controversial. In contrast to current concepts involving the autoxidation of nitric oxide ( • NO, nitrogen monoxide), we and others have proposed the formation of peroxynitrite (oxoperoxonitrate (1-)) as an essential intermediate. This requires low cellular fluxes of 'NO and superoxide ( • O 2 - ), for which model systems have been introduced. We here propose two new systems for nitros(yl)ation that avoid the shortcomings of previous models. Based on the thermal decomposition of 3-morpholinosydnonimine, equal fluxes of • NO and • O 2 - were generated and modulated by the …

Contributions of statistical modelling for the understanding of the nivo-glaciological dynamics of a small arctic glacial basin (Austre Lovén glacier…

2021

Since the middle of the 19th century, the Earth has experienced a climate shift marked by a high rise in temperature (+ 0.85 °C over the period 1880-2012). The Arctic is the region of the world that is warming the most rapidly, at a rate of 2 to 3 times faster than the global average. In this context, all components of the Arctic cryosphere are experiencing a change in their dynamics. Because of their direct links with the atmosphere, glaciers are among the best indicators of these climate variations. Like other glaciers on the globe, the glaciers of Svalbard, which cover 60% of the archipelago’s surface, have been retreating since the end of the Little Ice Age. This retreat, which is refle…

A 15-Year Perspective of the Fabry Outcome Survey

2016

Abstract The Fabry Outcome Survey (FOS) is an international long-term observational registry sponsored by Shire for patients diagnosed with Fabry disease who are receiving or are candidates for therapy with agalsidase alfa (agalα). Established in 2001, FOS provides long-term data on agalα safety/efficacy and collects data on the natural history of Fabry disease, with the aim of improving clinical management. The FOS publications have helped establish prognostic and severity scores, defined the incidence of specific disease variants and implications for clinical management, described clinical manifestations in special populations, confirmed the high prevalence of cardiac morbidity, and demon…

Long-term effectiveness of agalsidase alfa enzyme replacement in Fabry disease: A Fabry Outcome Survey analysis

2015

Outcomes from 5 years of treatment with agalsidase alfa enzyme replacement therapy (ERT) for Fabry disease in patients enrolled in the Fabry Outcome Survey (FOS) were compared with published findings for untreated patients with Fabry disease. Data were extracted from FOS, a Shire-sponsored database, for comparison with data from three published studies. Outcomes evaluated were the annualized rate of change in estimated glomerular filtration rate (eGFR) and left ventricular mass indexed to height (LVMI) as well as time to and ages at a composite morbidity endpoint and at death. FOS data were extracted for 740 treated patients who were followed for a median of ~ 5 years. Compared with no trea…

Effects of enzyme replacement therapy with agalsidase alfa on glomerular filtration rate in patients with Fabry disease: preliminary data

2007

Progressive deposition of globotriaosylceramide results in severe complications involving the kidney, heart and brain in both hemizygous male and heterozygous female patients with Fabry disease. Analysis of renal data from FOS - the Fabry Outcome Survey - suggests that enzyme replacement therapy with agalsidase alfa can significantly improve renal function in patients with Fabry disease, at least in those with a mild decrease in glomerular filtration rate, and may also be able to slow down the natural decline in renal function in patients with a moderate reduction in glomerular filtration rate. Conclusion: Initial results from the large cohort of patients within FOS indicate that treatment …

Pharmacokinetics of agalsidase alfa in male and female patients with Fabry disease

2007

Enzyme replacement therapy in Fabry disease: Comparison of agalsidase alfa and agalsidase beta

2008

Electrophysiological and neurochemical characterization of 7-nitroindazole and molsidomine acute and sub-chronic administration effects in the dopami…

2009

Nicotine, the main psychoactive ingredient in tobacco, stimulates dopamine (DA) function, increasing DA neuronal activity and DA release. DA is involved in both motor control and in the rewarding and reinforcing effects of nicotine; however, the complete understanding of its molecular mechanisms is yet to be attained. Substantial evidence indicates that the reinforcing properties of drugs of abuse, including nicotine, can be affected by the nitric oxide (NO) system, which may act by modulating central dopaminergic function. In this study, using single cell recordings in vivo coupled with microiontophoresis and microdialysis in freely moving animals, the role of NO signaling on the hyperacti…