نتایج جستجو برای: fabry
تعداد نتایج: 6280 فیلتر نتایج به سال:
The ATTA project has been created in order to laser cool and count single atoms of low level krypton contamination in xenon, motivated by the XENON Dark Matter program. An important component of the ATTA system is the laser, and its linewidth must be smaller than that of the atomic transition of the krypton atom to be trapped. An experiment was developed in order to measure this linewidth. A dr...
In this paper we present our recent works on optical injection of Fabry-Perot laser diode for application in access networks. The injection-locked Fabry-Perot laser diode is used as low-cost colorless transmitters for high-speed optical access exploiting wavelength-division-multiplexing technology. The modification of main characteristics of Fabry-Perot laser such as spectral properties, noise ...
AIM Fabry disease is caused by α-galactosidase A deficiency leading to accumulation of globotriaosylceramide (Gb3) in tissues. Clinical manifestations do not appear to correlate with total Gb3 levels. Studies examining tissue distribution of specific acyl chain species of Gb3 and upstream glycosphingolipids are lacking. MATERIAL & METHODS/RESULTS Thorough characterization of the Fabry mouse s...
Fabry disease is a lipid storage disorder resulting from mutations in the gene encoding the enzyme alpha-galactosidase A (alpha-gal A; EC ). We previously have demonstrated long-term alpha-gal A enzyme correction and lipid reduction mediated by therapeutic ex vivo transduction and transplantation of hematopoietic cells in a mouse model of Fabry disease. We now report marked improvement in the e...
Fabry disease (FD) is a lysosomal disorder leading to progressive systemic involvement, including microvascular damage that leads to neurological and cardiovascular disorders. We hypothesize that the latter could be documented at an early stage by performing a microcirculation study with nailfold capillaroscopy and evaluation of Raynaud phenomenon.The objective was to measure the prevalence of ...
Recent research has suggested a possible link between Parkinson's disease (PD) and Fabry disease. To test this relationship, we administered a self-report and family history questionnaire to determine the prevalence of PD in Fabry disease patients and family members with likely pathogenic alpha-galactosidase A (GLA) mutations. A total of 90 Fabry patients (77 from the online survey and 13 from ...
BACKGROUND Fabry disease is a rare X-linked disease arising from deficiency of alpha-galactosidase A. It results in early death related to renal, cardiac, and cerebrovascular disease, which are also important outcomes in patients with elevated blood pressure (BP). The prevalence of uncontrolled hypertension, as well as the effect of enzyme replacement therapy on BP, in patients with Fabry disea...
BACKGROUND Fabry disease. an X-linked deficiency of α-galactosidase A coded by the GLA gene, leads to intracellular globotriaosylceramide (GL-3) accumulation. Although less common than in males, chronic kidney disease, occurs in ∼ 15% of females. Recent studies highlight the importance of podocyte injury in Fabry nephropathy development and progression. We hypothesized that the greater the % of...
BACKGROUND Fabry disease is an inborn lysosomal storage disorder which is associated with small fiber neuropathy. We set out to investigate small fiber conduction in Fabry patients using pain-related evoked potentials (PREP). METHODS In this case-control study we prospectively studied 76 consecutive Fabry patients for electrical small fiber conduction in correlation with small fiber function ...
By spectrally slicing a single longitudinal-mode from a master weak-resonant-cavity Fabry-Perot laser diode with transient wavelength scanning and tracking functions, the broadened self-injection-locking of a slave weak-resonant-cavity Fabry-Perot laser diode is demonstrated to achieve bi-directional transmission in a 200-GHz array-waveguide-grating channelized dense-wavelength-division-multipl...
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