52 research outputs found

    Spatial Functional Characteristics of East Asian Patients with Occult Macular Dystrophy (Miyake disease); EAOMD Report No.2

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    PURPOSE: To describe the functional phenotypic features of East Asian patients with RP1L1-associated occult macular dystrophy (i.e., Miyake disease). DESIGN: An international multi-center retrospective cohort study. METHODS: Twenty-eight participants (53 eyes) with Miyake disease were enrolled at three centres: in Japan, China, and Korea. Ophthalmological examinations including spectral-domain optic coherence tomography (SD-OCT) and multifocal electroretinogram (mfERG) were performed. Patients were classified into three functional groups based on mfERG: Group 1, paracentral dysfunction with relatively preserved central/peripheral function; Group 2, homogeneous central dysfunction with preserved peripheral function; and Group 3, widespread dysfunction over the recorded area. Three functional phenotypes were compared in clinical parameters and SD-OCT morphological classification (severe phenotype, blurred/flat ellipsoid zone and absence of the interdigitation zone; mild phenotype, preserved ellipsoid zone). RESULTS: There were eight eyes in Group 1, 40 eyes in Group 2, and five eyes in Group 3. The patients in Group 1 showed significantly later onset (P=.005) and shorter disease duration (P=.002), compared with those in Group 2. All eight eyes in Group 1 showed the mild morphological phenotype, while 43/45 eyes in Groups 2 and 3 presented the severe phenotype, which identified a significant association between the functional grouping and the morphological classification (P<.001). CONCLUSIONS: A spectrum of functional phenotypes of Miyake disease was first documented with identifying three functional subtypes. Patients with paracentral dysfunction had the mildest phenotype, and those with homogeneous central or widespread dysfunction showed overlapping clinical findings with severe photoreceptor changes, suggesting various extents of visual impairment

    Functional Expression of the Extracellular Calcium Sensing Receptor (CaSR) in Equine Umbilical Cord Matrix Size-Sieved Stem Cells

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    The present study investigates the effects of high external calcium concentration ([Ca(2+)](o)) and the calcimimetic NPS R-467, a known calcium-sensing receptor (CaSR) agonist, on growth/proliferation of two equine size-sieved umbilical cord matrix mesenchymal stem cell (eUCM-MSC) lines. The involvement of CaSR on observed cell response was analyzed at both the mRNA and protein level.A large (>8 ”m in diameter) and a small (<8 ”m) cell line were cultured in medium containing: 1) low [Ca(2+)](o) (0.37 mM); 2) high [Ca(2+)](o) (2.87 mM); 3) NPS R-467 (3 ”M) in presence of high [Ca(2+)](o) and 4) the CaSR antagonist NPS 2390 (10 ”M for 30 min.) followed by incubation in presence of NPS R-467 in medium with high [Ca(2+)](o). Growth/proliferation rates were compared between groups. In large cells, the addition of NPS R-467 significantly increased cell growth whereas increasing [Ca(2+)](o) was not effective in this cell line. In small cells, both higher [Ca(2+)](o) and NPS R-467 increased cell growth. In both cell lines, preincubation with the CaSR antagonist NPS 2390 significantly inhibited the agonistic effect of NPS R-467. In both cell lines, increased [Ca(2+)](o) and/or NPS R-467 reduced doubling time values.Treatment with NPS R-467 down-regulated CaSR mRNA expression in both cell lines. In large cells, NPS R-467 reduced CaSR labeling in the cytosol and increased it at cortical level.In conclusion, calcium and the calcimimetic NPS R-467 reduce CaSR mRNA expression and stimulate cell growth/proliferation in eUCM-MSC. Their use as components of media for eUCM-MSC culture could be beneficial to obtain enough cells for down-stream purposes

    A case study of dioxin monitoring in and around an industrial waste incinerator in Korea

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    Many studies have been conducted that monitor and trace the sources of polychlorinated dibenzo-p-dioxin/furans (PCDD/Fs) by comparing congener patterns of environmental samples with those of possible sources. In this study, we measured PCDD/F concentrations and compared congener patterns of samples of various media found in and around an industrial waste incinerator in Korea, including stack gas, fly ash, bottom ash, ambient air, soil, pine needle, and human blood. We obtained reliable data on the relationship between the PCDD/F distributions in these sources and the environment, and thus found indicators with which to assess the impact of such sources on the surrounding environment. In addition, the difference between the levels of PCDD/Fs in the blood of short-term workers and long-term workers demonstrates that these workers are exposed to the compounds produced by the indicator. (c) 2004 Elsevier Ltd. All rights reserved.X1159sciescopu

    Facile synthesis and characterization of anatase TiO<inf>2</inf>/g-CN composites for enhanced photoactivity under UV–visible spectrum

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    © 2020 Elsevier Ltd For the purpose of atmospheric NO removal, anatase TiO2/g-CN photocatalytic composites were prepared by using a facile template-free calcination route in atmospheric conditions. Considerably fiscal NP400 and laboratory-grade melamine were used as the precursor of the composites. Additionally, samples were prepared with different wt. ratios of TiO2 and melamine by using two distinct calcination temperatures (550 °C/600 °C). The morphological attributes of the composites were assessed with X-ray diffraction, scanning and transmission electron microscopy, infrared spectroscopy, and X-ray photoelectron spectroscopy. Additionally, the optical traits were evaluated and compared using UV–visible diffuse reflectance spectroscopy and photoluminescence analysis. Finally, the photodegradation potentials for atmospheric NO by using the as-prepared composites were assessed under both UV and visible light irradiation. All the composites showed superior NO oxidation compared to NP400 and bulk g-CN. For the composites prepared by using the calcination temperature of 550 °C, the maximum NO removal was observed when the NP400 to melamine ratio was 1:2, irrespective of the utilized light irradiation type. Whereas for increased calcination temperature (600 °C), the maximum NO removal was observed at the precursor mix ratio of 1:3 (NP400:melamine). Successfully narrowed energy bandgaps were perceived in the as-prepared composites. Moreover, a subsequent drop in NO2 generation during NO oxidation was observed under both UV and visible light irradiation. Interestingly, higher calcination temperature during the synthesis of the catalysts has shown a significant drop in NO2 generation during the photodegradation of NO
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