نتایج جستجو برای: fabry
تعداد نتایج: 6280 فیلتر نتایج به سال:
This Letter addresses the challenging problems posed to the Kubo-Anderson (KA) theory by the discovery of intermittent resonant fluorescence with a nonexponential distribution of waiting times. We show how to extend the KA theory from aged to aging systems, aging for a very extended time period or even forever, being a crucial consequence of non-Poisson statistics.
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. In the heart, glycosphingolipids deposition causes progressive left ...
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 A activity may display all the characteris...
The general theory of a diaphragm fiber-optic sensor (DFOS) is proposed. We use a critical test to determine if a DFOS is based on Fabry-Perot interference or intensity modulation. By use of the critical test, this is the first design, to the best of our knowledge, of a purely Fabry-Perot DFOS, fabricated with microelectromechanical system technology, and characterized as an audible microphone ...
We experimentally demonstrate the phase relations of three-port gratings by investigating three-port coupled Fabry-Perot cavities. Two different gratings that have the same first-order diffraction efficiency but differ substantially in their second-order diffraction efficiency have been designed and manufactured. Using the gratings as couplers to Fabry-Perot cavities, we could validate the resu...
We report 16 new laser lines generated in a short-wavelength far-infrared Fabry-Perot laser cavity. The CO(2) pump laser is coupled into the far-infrared laser outside the far-infrared laser mode, and the cavity uses a 45 degrees adjustable output coupling mirror; this combination results in a low-loss Fabry-Perot cavity for wavelengths below 150 microm. The new lines are of medium and strong i...
The time for starting a patient with Fabry disease on enzyme replacement therapy is still a matter of debate, particularly when no overt classical clinical signs or symptoms are present. With respect to Fabry nephropathy, a dual problem coexists: the reluctance of many nephrologists to start enzyme replacement infusion until signs of renal disease appear as the appearance of proteinuria or an e...
In a previous paper we described details of the construction of stabilized 670-nm diode lasers for use in undergraduate physics laboratories. We report here a series of experiments that can be performed using the 670-nm diode laser, a homemade scanning Fabry-Perot cavity, a helium-neon laser, a simple photodiode, and a few pieces of electronics hardware. The experiments include: 1) an introduct...
Fabry disease is an X-linked recessive glycolipid storage disease. It is caused by deficiency of the lysosomal enzyme alpha-galactosidase A and leads to the accumulation of the enzyme substrate, globotriasylceramide (Gb3) in many tissues including endothelial cells, pericytes and smooth muscle cells of blood vessels, renal epithelial cells, cardiac myocytes and numerous neuronal cells. In this ...
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