Analysis Tools
vision/eye
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• at 9 months, some of the axon terminals of rod bipolar cells have retracted and/or appear swollen; the ribbon synapses are almost absent
• in contrast, the stratification pattern of cone bipolar cell axons and amacrine cell processes appear relatively unaffected
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• at PW5, many of the horizontal cell processes of mutant retinas show extensive sprouting into the ONL and INL
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• at 2 months, the double mutant retina exhibits a massive reduction of the outer nuclear layer, whereas the inner retina remains unaffected
• by 10 months, the outer retina has degenerated completely, whereas the inner retina still appears morphologically intact
• at later stages of retinal degeneration, the inner plexiform layer (IPL) is also affected
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• in double mutants, photoreceptor degeneration begins at ~PW4 and increases rapidly after PW7
• at PW10, the photoreceptor layer is reduced to one row of nuclei; by PW12, the entire ONL is missing in most parts of the retina
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• by PW4, the number of rod spherules is reduced to <50% of wild-type number
• double mutants have rod spherules with 2, 3, or more synaptic ribbons and larger numbers of postsynaptic elements
• at PW4, 45% of mutant rod spherules have 2 or more ribbons, whereas only 4% of wild-type shperules have more than 1 ribbon
• by PW5 and PW6, >80% of the surviving rod spherules contain 2 or more ribbons
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• double mutants exhibit a progressive degeneration of all photoreceptors within 3 months after birth
• notably, double mutants display normal expression of presynaptic markers and postsynaptic glutamate receptors in the outer plexiform layer (OPL) until postnatal week 7 (PW7)
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• at ~P35, scotopic ERGs confirm loss of both rod and cone-driven responses in double homozygotes
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nervous system
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• in double mutants, photoreceptor degeneration begins at ~PW4 and increases rapidly after PW7
• at PW10, the photoreceptor layer is reduced to one row of nuclei; by PW12, the entire ONL is missing in most parts of the retina
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• at 9 months, some of the axon terminals of rod bipolar cells have retracted and/or appear swollen; the ribbon synapses are almost absent
• in contrast, the stratification pattern of cone bipolar cell axons and amacrine cell processes appear relatively unaffected
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• at PW5, many of the horizontal cell processes of mutant retinas show extensive sprouting into the ONL and INL
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• by PW4, the number of rod spherules is reduced to <50% of wild-type number
• double mutants have rod spherules with 2, 3, or more synaptic ribbons and larger numbers of postsynaptic elements
• at PW4, 45% of mutant rod spherules have 2 or more ribbons, whereas only 4% of wild-type shperules have more than 1 ribbon
• by PW5 and PW6, >80% of the surviving rod spherules contain 2 or more ribbons
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• double mutants exhibit a progressive degeneration of all photoreceptors within 3 months after birth
• notably, double mutants display normal expression of presynaptic markers and postsynaptic glutamate receptors in the outer plexiform layer (OPL) until postnatal week 7 (PW7)
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