Analysis Tools
vision/eye
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• accumulation of vesicular and proteinaceous material is seen in the inner segment of 21 to 28 day old mice; this accumulation of material is not seen is mice older than 4 weeks of age
• accumulation of opsin (and some arrestin) within the cytoplasm of the inner segment is first seen at 21-24 days of age indicating that opsin mislocalization precedes photoreceptor death
• while opsin intracellular transport is impaired, distribution of other integral membrane proteins is normally localized to the outer segment
• no structural defect in the connecting cilium is seen
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• 10 week old mice show a range from no detectable reduction to loss of 80% of photoreceptors, indicating variable severity of photoreceptor degeneration
• many photoreceptor cells degenerate to opsin-containing fragments and opsin becomes aberrantly localized to the plasma membrane at late stages of photoreceptor degeneration
• photoreceptor degeneration is due to apoptotic photoreceptor cell death
• however, no cell loss is seen in 2 week old mice and no differences in the retinal ganglion or inner nuclear cell layers is seen
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nervous system
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• accumulation of vesicular and proteinaceous material is seen in the inner segment of 21 to 28 day old mice; this accumulation of material is not seen is mice older than 4 weeks of age
• accumulation of opsin (and some arrestin) within the cytoplasm of the inner segment is first seen at 21-24 days of age indicating that opsin mislocalization precedes photoreceptor death
• while opsin intracellular transport is impaired, distribution of other integral membrane proteins is normally localized to the outer segment
• no structural defect in the connecting cilium is seen
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• 10 week old mice show a range from no detectable reduction to loss of 80% of photoreceptors, indicating variable severity of photoreceptor degeneration
• many photoreceptor cells degenerate to opsin-containing fragments and opsin becomes aberrantly localized to the plasma membrane at late stages of photoreceptor degeneration
• photoreceptor degeneration is due to apoptotic photoreceptor cell death
• however, no cell loss is seen in 2 week old mice and no differences in the retinal ganglion or inner nuclear cell layers is seen
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