Analysis Tools
vision/eye
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• presence of large and irregular intercellular spaces between the photoreceptor outer segments
• large cysts and ectopic RPE cells are frequently seen in the photoreceptor outer segment layer of the retina
• alteration of the outer segment disk membranes
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• photoreceptor outer segments are 77%, 66% and 65% shorter at 3, 12, and 18 months of age, respectively
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• photoreceptor outer segments appear disorganized beginning at 1 month of age and onwards
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• retinal pigment epithelium (RPE) cells at 3 months of age are abnormally flat and elongated and often overlap with one another
• RPE cells have poorly developed apical microvilli and basal membrane infoldings and fewer phagosomes
• RPE cells have abnormal organelles such as large phagolysosomes and vacuoles and numerous lipid droplets
• cytoplasmic fragments, large cysts and myeloid bodies are seen on the apical side of the RPE
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• RPE exhibits an uneven distribution of melanin granules at P12 and fewer melanin granules within RPE cells
• melanin granule shape is irregular and typical fusiform granules are almost lacking
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• thickness of entire retinal pigment epithelium (RPE) is decreased at P12, and 3, 12, and 18 months of age, but not at E17.5 or P4
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• number of nuclear rows in the outer nuclear layer is 83%, 80% and 78% of controls at 3, 12, and 19 months of age, respectively
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• b-wave amplitudes in both flicker and photopic electroretinograms (ERGs) are reduced by about 50%, however no differences in the latency of b-waves
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• both a- and b-wave amplitudes are reduced by about 50% indicating reduced scotopic responses, however no differences in the latency of for the a- and b-waves
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• delay in dark adaptation after photo bleach
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pigmentation
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• retinal pigment epithelium (RPE) cells at 3 months of age are abnormally flat and elongated and often overlap with one another
• RPE cells have poorly developed apical microvilli and basal membrane infoldings and fewer phagosomes
• RPE cells have abnormal organelles such as large phagolysosomes and vacuoles and numerous lipid droplets
• cytoplasmic fragments, large cysts and myeloid bodies are seen on the apical side of the RPE
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• RPE exhibits an uneven distribution of melanin granules at P12 and fewer melanin granules within RPE cells
• melanin granule shape is irregular and typical fusiform granules are almost lacking
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• thickness of entire retinal pigment epithelium (RPE) is decreased at P12, and 3, 12, and 18 months of age, but not at E17.5 or P4
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nervous system
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• presence of large and irregular intercellular spaces between the photoreceptor outer segments
• large cysts and ectopic RPE cells are frequently seen in the photoreceptor outer segment layer of the retina
• alteration of the outer segment disk membranes
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• photoreceptor outer segments are 77%, 66% and 65% shorter at 3, 12, and 18 months of age, respectively
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• photoreceptor outer segments appear disorganized beginning at 1 month of age and onwards
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