Abstract
In humans cone photoreceptors are responsible for high-resolution colour vision. A variety of retinal diseases can compromise cone viability, and, at present, no satisfactory treatment options are available. Here, we present data towards establishing a reliable, high-throughput assay system that will facilitate the search for cone neuroprotective compounds using the murine-photoreceptor cell line 661 W. To further characterize 661 W cells, a retinal marker study was performed, followed by the induction of cell death using paradigms over-activating cGMP-dependent protein kinase G (PKG). We found that 661 W cells may be used to mimic specific aspects of cone degeneration and may thus be valuable for future compound screening studies.
Keywords:
Achromatopsia; CNG channel; Neurodegeneration; cGMP; cpfl1; rd1.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Biomarkers
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Cell Line, Tumor
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Cyclic GMP / metabolism
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Cyclic GMP-Dependent Protein Kinases / drug effects
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Cyclic GMP-Dependent Protein Kinases / physiology*
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Cyclic Nucleotide Phosphodiesterases, Type 6 / deficiency
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Drug Evaluation, Preclinical / methods*
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Enzyme Activation / drug effects
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Eye Proteins / analysis
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Eye Proteins / physiology*
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High-Throughput Screening Assays*
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Mice
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Mice, Knockout
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Neuroprotective Agents / isolation & purification*
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Neuroprotective Agents / pharmacology
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Organ Specificity
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Phosphodiesterase Inhibitors / pharmacology
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Purinones / pharmacology
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Retinal Cone Photoreceptor Cells / cytology
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Retinal Cone Photoreceptor Cells / enzymology*
Substances
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Biomarkers
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Eye Proteins
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Neuroprotective Agents
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Phosphodiesterase Inhibitors
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Purinones
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Cyclic GMP-Dependent Protein Kinases
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Cyclic Nucleotide Phosphodiesterases, Type 6
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zaprinast
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Cyclic GMP