vision/eye
• lipid peroxidation is increased in inner segment photoreceptor cells at P12, and a modest increase is seen in the other layers of the retina including the inner nuclear layer and ganglion cell layer
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• 80% decrease in the number of connecting cilia in photoreceptor cells
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• mitochondrial area in inner segment of photoreceptor cells is decreased indicating a decrease in mitochondrial biomass at P11
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• the outer segment layer is very thin
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• the outer segment is disorganized and consists of cell membranes that branch and form a large web-like network structure without apparent disc rim and an abnormally wide intra- and inter-outer segment membrane space
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• milder thinning or degeneration of the inner nuclear layer
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• rapid loss of the outer nuclear layer
• an increasing number of TUNEL+ cells are seen in the outer nuclear layer after P12
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• mice show rapid and massive retinal degeneration after P12 with none of the photoreceptor cells retained by P21
• cone photoreceptor cells are lost earlier than rod photoreceptor cells
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nervous system
• 80% decrease in the number of connecting cilia in photoreceptor cells
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• mitochondrial area in inner segment of photoreceptor cells is decreased indicating a decrease in mitochondrial biomass at P11
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• the outer segment layer is very thin
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• the outer segment is disorganized and consists of cell membranes that branch and form a large web-like network structure without apparent disc rim and an abnormally wide intra- and inter-outer segment membrane space
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cellular
• 80% decrease in the number of connecting cilia in photoreceptor cells
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• mitochondrial DNA replication in the whole retina is lower
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