Analysis Tools
vision/eye
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• expression analysis indicates abnormalities in synaptic ribbons as early as 5 weeks of age that become more severe with age
• ribbon size is reduced
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• number and size of cone pedicles appear to be decreased at 5 weeks of age and this decrease progresses with age
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• decrease in the number of M opsin+ cones in the superior retina
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• progressive thinning starting at 6 months of age
• thickness is reduced by about 30% in the superior retina at 6 months of age but no change is detected in the inferior retina
• by 1 year of age the entire retina is affected
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• progressive retinal degeneration starting by 6 months of age, with the super retina more affected than the inferior retina
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• decrease in the b wave amplitude in mice at 5 weeks, 6 months and 1 year of age
• abnormalities progress with age and more severely affect photopic b wave amplitudes compared to scotopic b waves
• at 1 year of age, the b wave amplitude is less than 10% that of wild-type control littermates
• a-wave amplitudes decrease with age starting after 5 weeks of age and the photopic response decreases more rapidly than the scotopic response
• at 6 months and 1 year of age, the a wave amplitude is 40% and less than 10% that of controls, respectively
• latency (time from onset of stimulus to beginning of the a wave) and implicit time (time from the onset of light stimulus to the peak of the b wave) are increased from 6 months of age on
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• decrease in the b wave amplitude in mice at 5 weeks, 6 months and 1 year of age
• abnormalities progress with age and more severely affect photopic b wave amplitudes compared to scotopic b waves
• a-wave amplitudes decrease with age starting after 1 year of age, when it is decreased 43-56% of control, and the photopic response decreases more rapidly than the scotopic response
• latency (time from onset of stimulus to beginning of the a wave) and implicit time (time from the onset of light stimulus to the peak of the b wave) are increased from 6 months of age on
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growth/size/body
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• decreased by about 24% compared to controls
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behavior/neurological
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• unstable gait
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homeostasis/metabolism
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• increase in the sensitivity of retina to light damage
• exposure to 10,000-lx cool, white fluorescent light for 2 h followed by 2 weeks in the dark results in increased loss of nuclear layers in the ONL with the superior retina showing more damage than the inferior retina
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nervous system
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• expression analysis indicates abnormalities in synaptic ribbons as early as 5 weeks of age that become more severe with age
• ribbon size is reduced
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• number and size of cone pedicles appear to be decreased at 5 weeks of age and this decrease progresses with age
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• decrease in the number of M opsin+ cones in the superior retina
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• expression analysis indicates abnormalities in photoreceptor synaptic ribbons as early as 5 weeks of age that become more severe with age
• photoreceptor synaptic ribbon size is reduced
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integument
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• increase in the sensitivity of retina to light damage
• exposure to 10,000-lx cool, white fluorescent light for 2 h followed by 2 weeks in the dark results in increased loss of nuclear layers in the ONL with the superior retina showing more damage than the inferior retina
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