Results 11 to 20 of about 134,977 (268)
Journal of Cardiovascular Ultrasound, 2013 Fabry disease is a progressive X-linked disorder of glycosphingolipid metabolism caused by a deficiency of the α-galactosidase lysosomal enzyme. The partial or complete deficiency of the lysosomal enzyme leads to an accumulation of neutral glycosphingolipids in the vascular endothelium and visceral tissues throughout the body.
Yoon, Jae Yong +9 more
openaire +2 more sources
Long-time optical transmittance measurements of silica nanofibers [PDF]
EPJ Web of Conferences, 2020 We perform long-time measurements of the optical silica transmittance during several months in different environments and with different nanofiber lengths.
Bouhadida Maha, Lebrun Sylvie
doaj +1 more source
Backaction in metasurface etalons [PDF]
, 2016 We consider the response of etalons created by a combination of a conventional mirror and a metasurface, composed of a periodic lattice of metal scatterers with a resonant response.
A. Femius Koenderink +5 more
core +11 more sources
Journal of the European Optical Society-Rapid Publications, 2023 Line-field Confocal Optical Coherence Tomography (LC-OCT) is an imaging modality based on a combination of time-domain optical coherence tomography and reflectance confocal microscopy.
Waszczuk Léna +3 more
doaj +1 more source
Orphanet Journal of Rare Diseases, 2010 Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal α-galactosidase A activity. FD is pan-ethnic and the reported annual incidence of 1 in 100,000 may underestimate the true prevalence of the disease. Classically affected hemizygous males, with no residual α-galactosidase
openaire +3 more sources
Light Extraction and Brightness Enhancement of Luminescent Rectangular Slabs
Advanced Photonics Research, 2022 The extraction efficiency and the brightness are critical parameters to increase the performance of luminescent rectangular slabs such as scintillators, luminescent concentrators, or diamonds with NV centers.
Pierre Pichon +6 more
doaj +1 more source
Radiative heat exchange driven by acoustic modes between two solids at the atomic scale [PDF]
EPJ Web of Conferences, 2023 In the near field (for separation distances smaller than the thermal wavelength, of the order of some microns at ambient temperature), the radiative heat flow between two solids at different temperatures can exceed the blackbody limit by several orders ...
Gómez Viloria Mauricio +4 more
doaj +1 more source
Characterization of early disease status in treatment-naive male paediatric patients with Fabry disease enrolled in a randomized clinical trial. [PDF]
, 2015 Trial designThis analysis characterizes the degree of early organ involvement in a cohort of oligo-symptomatic untreated young patients with Fabry disease enrolled in an ongoing randomized, open-label, parallel-group, phase 3B clinical trial.MethodsMales
An Haack, Kristina +17 more
core +8 more sources
Electrical generation of visible surface plasmon polaritons by a nanopillars antenna array
APL Photonics, 2021 Electrical excitation of surface plasmon polaritons by inelastic tunneling electrons has been put forward as a potential nanosource that can be used in a variety of on-chip optoelectronic applications.
Cheng Zhang +4 more
doaj +1 more source
Ultrahigh finesse Fabry-Perot superconducting resonator [PDF]
, 2007 We have built a microwave Fabry-Perot resonator made of diamond-machined copper mirrors coated with superconducting niobium. Its damping time (Tc = 130 ms at 51 GHz and 0.8 K) corresponds to a finesse of 4.6 x 109, the highest ever reached for a ...
Bernu, Julien +12 more
core +4 more sources