915 research outputs found

    Electroretinography

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    What Happened to the ‘Development’ in Sustainable Development? Business Guidelines Two Decades After Brundtland

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    ABSTRACTOver 20 years ago Our Common Future presented a conceptualization and explanation of the concept of sustainable development. Since then, numerous alternative definitions of the concept have been offered, of which at least some are exclusive to each other. At the same time, the role of business in the transition to sustainable development has increasingly received attention. Bringing these two trends in sustainable development together, this paper returns to the Brundtland version of the concept to examine to what extent the original principles of sustainable development are still embedded within key business guidelines, namely the UN Global Compact, the OECD Guidelines for Multinational Enterprises, the ICC Business Charter for Sustainable Development, the CAUX Principles, the Global Sullivan Principles and the CERES Principles. The findings suggest that these business guidelines tend to emphasize environmental rather than social aspects of sustainable development, in particular to the detriment of the original Brundtland prioritization of the needs of the poorest. Furthermore, the attention to environmental aspects stresses win–win situations and has a clear managerialist focus; whereas more conceptual environmental issues concerning systems interdependencies, critical thresholds or systemic limits to growth find little attention. The normative codes and principles targeted at the private sector therefore not only add another voice to the multiple discourses on sustainable development but also contribute to a reinterpretation of the original agenda set by Brundtland towards conceptualizations of sustainable development around the needs of industrialized rather than developing countries. Copyright © 2011 John Wiley &amp; Sons, Ltd and ERP Environment.</jats:p

    Patient-Specific iPSC-Derived RPE for Modeling of Retinal Diseases

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    Inherited retinal diseases, such as age-related macular degeneration and retinitis pigmentosa, are the leading cause of blindness in the developed world. Currently, treatments for these conditions are limited. Recently, considerable attention has been given to the possibility of using patient-specific induced pluripotent stem cells (iPSCs) as a treatment for these conditions. iPSCs reprogrammed from adult somatic cells offer the possibility of generating patient-specific cell lines in vitro. In this review, we will discuss the current literature pertaining to iPSC modeling of retinal disease, gene therapy of iPSC-derived retinal pigmented epithelium (RPE) cells, and retinal transplantation. We will focus on the use of iPSCs created from patients with inherited eye diseases for testing the efficacy of gene or drug-based therapies, elucidating previously unknown mechanisms and pathways of disease, and as a source of autologous cells for cell replacement

    Late-stage rescue of visually guided behavior in the context of a significantly remodeled retinitis pigmentosa mouse model

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    Patients with progressive neurodegenerative disorder retinitis pigmentosa (RP) are diagnosed in the midst of ongoing retinal degeneration and remodeling. Here, we used a Pde6b-deficient RP gene therapy mouse model to test whether treatment at late disease stages can halt photoreceptor degeneration and degradative remodeling, while sustaining constructive remodeling and restoring function. We demonstrated that when fewer than 13% of rods remain, our genetic rescue halts photoreceptor degeneration, electroretinography (ERG) functional decline and inner retinal remodeling. In addition, in a water maze test, the performance of mice treated at 16 weeks of age or earlier was indistinguishable from wild type. In contrast, no efficacy was apparent in mice treated at 24 weeks of age, suggesting the photoreceptors had reached a point of no return. Further, remodeling in the retinal pigment epithelium (RPE) and retinal vasculature was not halted at 16 or 24 weeks of age, although there appeared to be some slowing of blood vessel degradation. These data suggest a novel working model in which restoration of clinically significant visual function requires only modest threshold numbers of resilient photoreceptors, halting of destructive remodeling and sustained constructive remodeling. These novel findings define the potential and limitations of RP treatment and suggest possible nonphotoreceptor targets for gene therapy optimization

    Erythropoetin receptor expression in the human diabetic retina

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    <p>Abstract</p> <p>Background</p> <p>Recent evidence suggests erythropoietin (<it>EPO</it>) and the erythropoietin receptor (<it>EPOR</it>) may play a direct role in the pathogenesis of diabetic retinopathy. Better characterization of the <it>EPO-EPOR </it>signaling system in the ischemic retina may offer a new therapeutic modality for ischemic ophthalmic diseases. This study was performed to identify <it>EPOR </it>mRNA expression in the human diabetic eye.</p> <p>Findings</p> <p><it>EPOR </it>antisense RNA probes were validated on human pancreas tissue. In the normal eye, <it>EPOR </it>was expressed in the retinal ganglion cell layer. Minimal expression was observed in the inner and outer nuclear layer. Under conditions of diabetic retinopathy, <it>EPOR </it>expression shifted to photoreceptor cells. Increased expression was also observed in the peripheral retina.</p> <p>Conclusion</p> <p><it>EPOR </it>expression may be a biomarker or contribute to disease mechanisms in diabetic retinopathy.</p

    Measuring Trust in Government: A Hong Kong Perspective

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    Trust and legitimacy occupy a central position in contemporary discourse surrounding the process of environmental reform in late-modern societies. This study examines dimensions of trust from stakeholders and uses a group process to enrich the data describing and explaining the reasons behind a possible ‘trust deficit’ in the context of environmental governance and policy making in Hong Kong. Results from focus groups indicate that trust in government with regard to environmental issues is generally very low. Factors include poor leadership, a rather out-dated mindset of the government, inflexible government structures, inconsistent governance, misplaced knowledge and expertise in the government and its reluctance to create dialogue or communication. Stakeholders suggested that to enhance public trust in the government, the government needed to develop stronger leadership, reform government structure, improve communication on environmental issues to the public, take input from the community more seriously and make better use of regulation and provide incentives for environmental protection

    Unilateral electronegative ERG in a presumed central retinal artery occlusion

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    A unilateral electronegative electroretinogram (ERG) was seen in a 94-year-old man with presumed central retinal artery occlusion. Goldmann perimetry revealed central scotoma in the right eye and no abnormalities in the left eye. Full-field ERG in the right eye described a reduction of the b-wave with a relative preservation of the a-wave which is characteristic of electronegative ERG. Hence, our case illustrates that ERG testing is essential for the work-up of individuals with suspected retinal vascular disorders

    Precision Medicine: Genetic Repair of Retinitis Pigmentosa in PatientDerived Stem Cells

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    Induced pluripotent stem cells (iPSCs) generated from patient fibroblasts could potentially be used as a source of autologous cells for transplantation in retinal disease. Patient-derived iPSCs, however, would still harbor disease-causing mutations. To generate healthy patient-derived cells, mutations might be repaired with new gene-editing technology based on the bacterial system of clustered regularly interspersed short palindromic repeats (CRISPR)/Cas9, thereby yielding grafts that require no patient immunosuppression. We tested whether CRISPR/Cas9 could be used in patient-specific iPSCs to precisely repair an RPGR point mutation that causes X-linked retinitis pigmentosa (XLRP). Fibroblasts cultured from a skin-punch biopsy of an XLRP patient were transduced to produce iPSCs carrying the patient’s c.3070G>T mutation. The iPSCs were transduced with CRISPR guide RNAs, Cas9 endonuclease, and a donor homology template. Despite the gene’s repetitive and GC-rich sequences, 13% of RPGR gene copies showed mutation correction and conversion to the wild-type allele. This is the first report using CRISPR to correct a pathogenic mutation in iPSCs derived from a patient with photoreceptor degeneration. This important proof-of-concept finding supports the development of personalized iPSC-based transplantation therapies for retinal disease
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