Search results for "PLD"

The role of registries in rare genetic lipid disorders: Review and introduction of the first global registry in lipoprotein lipase deficiency

2017

International audience; A good understanding of the natural history of rare genetic lipid disorders is a pre-requisite for successful patient management. Disease registries have been helpful in this regard. Lipoprotein Lipase Deficiency (LPLD) is a rare, autosomal-recessive lipid disorder characterized by severe hypertriglyceridemia and a very high risk for recurrent acute pancreatitis, however, only limited data are available on its natural course. Alipogene tiparvovec (Glybera (R)) is the first gene therapy to receive Marketing Authorization in the European Union; GENIALL (GENetherapy In the MAnagement of Lipoprotein Lipase Deficiency), a 15-year registry focusing on LPLD was launched in …

Indium Tin Oxide Photoablation: Spectroscopic Analysis of the Plume

1999

Deciphering the Nonsense Readthrough Mechanism of Action of Ataluren: An in Silico Compared Study

2019

Ataluren was reported to suppress nonsense mutations by promoting the readthrough of premature stop codons, although its mechanism of action (MOA) is still debated. The likely interaction of Ataluren with CFTR-mRNA has been previously studied by molecular dynamics. In this work we extended the modeling of Ataluren's MOA by complementary computational approaches such as induced fit docking (IFD), quantum polarized ligand docking (QPLD), MM-GBSA free-energy calculations, and computational mutagenesis. In addition to CFTR-mRNA, this study considered other model targets implicated in the translation process, such as eukaryotic rRNA 18S, prokaryotic rRNA 16S, and eukaryotic Release Factor 1 (eRF…

Resistive switching behaviour in ZnO and VO 2 memristors grown by pulsed laser deposition

2014

The resistive switching behaviour observed in microscale memristors based on laser ablated ZnO and VO 2 is reported. A comparison between the two materials is reported against an active device size. The results show that devices up to 300 × 300 μm 2 exhibit a memristive behaviour regardless of the device size, and 100 × 100 μm 2 ZnO-based memristors have the best resistance off/on ratio.

Upregulation of Phospholipase D Expression and Activation in Ventricular Pressure-Overload Hypertrophy

2005

Evidence for a role of phospholipase D (PLD) in cellular proliferation and differentiation is accumulating. We studied PLD activity and expression in normal and hypertrophic rat and human hearts. In rat heart, abdominal aortic banding (constriction to 50% of original lumen) caused hypertrophy in the left ventricle (as shown by weight index and ANP expression) by about 15% after 30 days without histological evidence of fibrosis or signs of decompensation and in the right ventricle after 100 days. The hypertrophy was accompanied by small increases of basal PLD activity and strong potentiation of stimulated PLD activity caused by 4β-phorbol-12β,13α-dibutyrate (PDB) and by phenylephrine. The mR…

In situ monitoring of pulsed laser indium–tin-oxide film deposition by optical emission spectroscopy

2001

We performed optical emission spectroscopy to monitor the plasma produced during the ablation of indium-tin-oxide targets under different oxygen pressure conditions using a pulsed UV laser. Molecular bands of InO were identified in the fluorescent spectra produced by pulsed laser ablation. InO line monitoring allowed obtaining the optimal conditions for good-quality ITO film deposition. We demonstrated that it is possible to correlate InO line spectroscopic parameters with the conditions required to fabricate a high-conductivity and high-transparent ITO thin film. In particular, low resistivity (10-4 to 10-3 Ω cm) was obtained in films deposited at room temperature by regulating oxygen pres…

Fabrication and characterization of microscale HfO2-based Memristors

2017

Memristors are metal/insulator/metal devices whose resistance can be switched between two different states (i.e. the low resistive state LRS, and the high resistive state, HRS) by applying a proper voltage value over the two metal contacts [1], [2]. Their simple structure makes memristors prone to extreme down scaling and 3-D stacking potentiality, and excellent compatibility with the complementary metal-oxide-semiconductor (CMOS) technology. Moreover, because of their low power consumption and high speed, memristors are rightly considered the elemental bricks for a next generation of high-density nonvolatile memories. HfO2 has attracted much attention as an oxide material for memristor app…

Polycrystalline MoO3 films fabricated by pulsed laser deposition for infrared polarization manipulation

2022

We performed infrared optical characterization of polycrystalline MoO3 films deposited by pulsed laser deposition on fused silica substrates. Several samples have been fabricated using different parameters such as temperature and oxygen pressure. Our analysis shows that under appropriate fabrication conditions it is possible to obtain a dominant α-phase film, with a well-defined, normal to surface (z-axis) orientation. These results are confirmed by reflection spectra performed at 45° incidence angle revealing a strong modulation of the sharp z-phonon Reststrahlen band as a function of the incident field linear polarization.

Deposition of High Quality Indium Tin Oxide Films by Monitoring InO Emission Lines

2001

Issues Affecting Quality of Life and Disease Burden in Lipoprotein Lipase Deficiency (Lpld) - First Step Towards a Pro Measure in Lpld

2015

Objectives: LPLD is an ultra-orphan genetic lipid disorder (prevalence 1-2/million). It is associated with severe hypertriglyceridemia and an increased risk of acute pancreatitis. Other manifestations include eruptive xanthoma, fatigue, difficulty with concentrating and cardiopulmonary symptoms. Associated symptoms, complications and the fat-restricted diet affect Quality of Life (QOL). Currently no disease-specific measure exists to assess QOL and the burden of LPLD. As part of post approval commitments of alipogene tiparvovec, regulatory bodies requested the development of a reliable measure of QOL in LPLD. This study evaluates existing EORTC questionnaires QLQ-C30 and QLQ-PAN26, and a ne…