Search results for "FCS"
showing 10 items of 13 documents
Effectiveness and safety of lomitapide in a patient with familial chylomicronemia syndrome
2020
Background: Familial chylomicronemia syndrome (FCS) is characterized by severe fasting hypertriglyceridemia, abdominal pain, and recurrent acute pancreatitis. Available triglyceride-lowering drugs are insufficient to avoid pancreatitis. Therefore, there is a significant unmet medical need for effective triglyceride-lowering drugs for patients with FCS. Case report: We report the second case of a patient with FCS and recurrent pancreatitis treated with lomitapide. Lomitapide treatment resulted in a reduction of fasting TG levels from 2897 mg/dL (32.71 mmol/L) to an average of 954 mg/dL (10.77 mmol/L) on the 30 mg lomitapide equating to a 67% reduction from baseline. After 26 months of lomita…
A methodological protocol for Annex I Habitats monitoring: the contribution of Vegetation science.
2016
The methodological foundations of the recently published Manual for Annex I Habitats monitoring in Italy, edited by the Italian Institute for Environmental Protection and Research (ISPRA) with the scientific support of the Italian Society for Vegetation Science (SISV), are here presented, discussed and related to the most consolidated and acknowledged scientific advances in the field of Vegetation science. The proposed methodologies aim at offering simple, yet effective, protocols and procedures towards a harmonized data collection, by way of standardized and shared technical tools, resulting in comparable evaluations of the conservation status of Annex I Habitats. The methodological framew…
Stronger proprioceptive BOLD-responses in the somatosensory cortices reflect worse sensorimotor function in adolescents with and without cerebral pal…
2020
Graphical abstract
On-site Experimental Study of HCFC-22 Substitution with HFCs Refrigerants
2012
Abstract The European Regulation no 2037/2000 has banned manufacturing HCFC refrigerants from January 1st 2010, although its use is allowed up to 2015 if the fluids come from a recycling process. This situation creates the need for developing new working fluids to replace the HCFC in the refrigeration plants now in operation. Among all the HCFCs the R22 is the most widely used in a wide range of applications, especially in air conditioning. This paper presents the results of an experimental analysis comparing the performance of a vapour compression refrigerating unit operating with R22, and its performance in comparison to some HFCs fluids, substituting the former. In particular, the plant …
Cardio-facio-cutaneous syndrome and gastrointestinal defects: report on a newborn with 19p13.3 deletion including the MAP 2 K2 gene
2022
Abstract Background Cardio-facio-cutaneous syndrome (CFCS) belongs to RASopathies, a group of conditions caused by mutations in genes encoding proteins of the rat sarcoma/mitogen-activated protein kinase (RAS/MAPK) pathway. It is a rare syndrome, with about 300 patients reported. Main clinical manifestations include facial dysmorphisms, growth failure, heart defects, developmental delay, and ectodermal abnormalities. Mutations (mainly missense) of four genes (BRAF, MAP 2 K1, MAP 2 K2, and KRAS) have been associated to CFCS. However, whole gene deletions/duplications and chromosomal microdeletions have been also reported. Specifically, 19p13.3 deletion including MAP 2 K2 gene are responsible…
Ionic liquid crystals based on 3-perfluoalkyl-1,2,4-triazol-4-ium salts
2016
Liquid-crystalline ionic liquids (LC-ILs) are a class of organic materials that of great current interest: they are defined as organic salts that possess the properties of two interesting kinds of material – ionic liquids (ILs) and liquid crystals (LCs). LC-ILs combine many interesting features of ILs (e.g. low volatility and the ability to dissolve solutes with a range of polarities) as well as many attractive properties of LCs (e.g. their intrinsic order and anisotropy). This provides unique opportunities that can be exploited in many different fields, for example their use as solvents for extraction processes as well as electrolytes for batteries, fuel cells, and dye-sensitised solar cel…
In Vitro–In Vivo Fluctuation Spectroscopies
2010
Fluorescence correlation spectroscopy (FCS) was first developed for biophysical studies in analogy with photon scattering correlation spectroscopy. Although it is mainly devoted to the study of freely diffusing particles, FCS is actually able to discern between different kinds of motions, such as diffusion, anomalous diffusion, or drift motions. The frontier application of FCS nowadays is in medical studies both within cells and on the cell membranes, and in the investigation of single molecules in solid matrices. In this field, FCS originated also image correlation spectroscopy methods. The whole field can be unified under the name of fluorescence fluctuation spectroscopy (FFS). We present…
Evaluation of a new Cr-free alloy as interconnect material for hydrogen production by high temperature water vapour electrolysis: Study in cathode at…
2012
International audience; For economic and ecological reasons, hydrogen is considered as a major energetic vector for the future. Hydrogen production via high temperature water vapour electrolysis (HTE) is a promising technology. A major technical difficulty related to high temperature water vapour electrolysis is the development of interconnects working efficiently for a long period. Working temperature of 800 degrees C enables the use of metallic materials as interconnects. High temperature corrosion behaviour and electrical conductivity of a new Cr-free Fe-Ni-Co alloy were tested in cathode atmosphere (H-2/H2O) at 800 degrees C. The alloy exhibits a poor oxidation resistance but an excelle…
Structure, interdomain dynamics, and pH-dependent autoactivation of pro-rhodesain, the main lysosomal cysteine protease from African trypanosomes
2021
AbstractRhodesain is the lysosomal cathepsin L-like cysteine protease ofT. brucei rhodesiense, the causative agent of Human African Trypanosomiasis. The enzyme is essential for the proliferation and pathogenicity of the parasite as well as its ability to overcome the blood-brain barrier of the host. Lysosomal cathepsins are expressed as zymogens with an inactivating pro-domain that is cleaved under acidic conditions. A structure of the uncleaved maturation intermediate from a trypanosomal cathepsin L-like protease is currently not available. We thus established the heterologous expression ofT. brucei rhodesiensepro-rhodesain inE. coliand determined its crystal structure. The trypanosomal pr…
Structural and electrochemical investigation on re-cast Nafion membranes for polymer electrolyte fuel cells (PEFCs) application
2006
Abstract Starting from a 5 wt% ion power Nafion® solution and dissolving the dry residue in a suitable solvent (10 wt% solution in dimethylacetammide), re-cast Nafion films were developed for polymer electrolyte fuel cells (PEFCs) application. The used Doctor-Blade casting technique allowed to prepare scaled-up membranes comparable to the commercial Nafion ones with good mechanical resistance, thickness homogeneity and an excellent reproducibility. The differences between the re-cast membrane nanostructure and that of the commercial Nafion were investigated by synchrotron small-angle X-ray scattering. The obtained re-cast membrane shows at ambient conditions a semi-crystalline structure wit…