Analysis Tools
vision/eye
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• at P45 (i.e. prior to onset of photoreceptor degeneration), neural retina contains significantly lower levels of flavin adenine dinucleotide (FAD, ~46.1%), flavin mononucleotide (FMN, ~45%), and riboflavin (RF, ~32%) relative to wild-type retinas
• at P120, but not at P45, neural retina shows a significant reduction in ATP level relative to wild-type retinas
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• at P240, but not at P60, mice show a ~45% reduction in the number of cone photoreceptor cells relative to wild-type controls
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• at P240, but not at P60, mice show a ~36.8% reduction in the number of rod photoreceptor cells relative to wild-type controls
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• TEM analysis revealed minor but obvious morphological changes in the central area of the outer segment (OS) at all ages examined (P60, P120, and P240)
• however, no major structural defects are seen at the OS/RPE interface or at the OS/IS junction
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• abnormally low levels of FAD and FMN in the neural retina lead to progressive degeneration of both rods and cones
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• at P120 and P240, mice show a ~37.5% and ~52.8% reduction, respectively, in scotopic a-wave amplitude relative to wild-type controls
• however, no significant changes are observed at P30 and P60
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• at P120 and P240, mice show a ~25%% and ~32% reduction, respectively, in photopic b-wave amplitude relative to wild-type controls
• however, no significant changes are observed at P30 and P60
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• mice show a late-onset but progressive decline in photopic ERG responses
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• mice show a late-onset but progressive decline in scotopic ERG responses
• at P120, but not at P60, mice show a significant reduction in scotopic a-wave and b-wave amplitudes at higher light intensities relative to wild-type controls
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nervous system
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• at P240, but not at P60, mice show a ~45% reduction in the number of cone photoreceptor cells relative to wild-type controls
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• at P240, but not at P60, mice show a ~36.8% reduction in the number of rod photoreceptor cells relative to wild-type controls
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• TEM analysis revealed minor but obvious morphological changes in the central area of the outer segment (OS) at all ages examined (P60, P120, and P240)
• however, no major structural defects are seen at the OS/RPE interface or at the OS/IS junction
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• abnormally low levels of FAD and FMN in the neural retina lead to progressive degeneration of both rods and cones
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