vision/eye
N |
• no obvious alterations in ONL thickness and cell numbers of bipolar, horizontal, ganglion, amacrine cells or Muller glial cells at P21
• normal array of the 9 + 0 microtubule doublets in transverse sections of the connecting cilium
• inner segments develop without severe defects at P21
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• significantly reduced number of cone photoreceptor cells at P30
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• significantly reduced number of rod photoreceptor cells at P30
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• signal intensities of bipolar dendritic markers are reduced at P14
• synaptic ribbons and tips of bipolar dendrites are ectopically localized in the ONL at P21 and P30
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• cone bipolar cell dendrites abnormally extend together with horizontal processes into the ONL
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• synaptic ribbons and tips of horizontal processes are ectopically localized in the ONL at P21 and P30
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• at P21, outer segments of both rod and cone photoreceptors are significantly shorter than wild-type
• however, the disk structure of outer segments remains normal
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• outer segments of S- and M-cone photoreceptors are markdely shortened at both P14 and P30
• the length of M-cone photoreceptor outer segments is significantly shorter at P21
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• outer segments of rod photoreceptors are shortened at both P14 and P30
• the length of rod photoreceptor outer segments is significantly shorter at P21
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• progressive photoreceptor degeneration
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• at P21, photoreceptor synaptic ribbons are frequently scattered into the ONL
• ectopic ribbons are more frequently found in the ONL at P30 than at P14 and P21
• ectopic ribbons often localize at the tip of bipolar cell dendrites and horizontal cell processes at P21 and P30
• at P21, EM revealed that a typical triad structure with bipolar dendritic tips is observed at low frequency
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• decreased thickness of the ONL starting at P30
• ONL thickness reduced to 27% of that in wild-type controls at 6 months of age
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• progressive degeneration of the ONL after P30, becoming severe at 6 months of age
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• the number of photoreceptor synaptic ribbons is reduced and aberrant and irregularly shaped ribbons are frequently observed in the OPL at P14
• the numbers of both synaptic ribbons and synaptic puncta are significantly reduced in the OPL at P21
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• markedly decreased amplitudes in a-waves under scotopic conditions at P30
• markedly decreased amplitudes in a-waves at high stimulus (0.5 and 1.0 log cd-s/m2) under photopic conditions at P30
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• markedly decreased amplitude in b-wave under scotopic conditions at P30
• almost complete loss of b-wave amplitude at high stimulus (0 to 1.0 log cd-s/m2) under photopic conditions at P30
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nervous system
• significantly reduced number of cone photoreceptor cells at P30
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• significantly reduced number of rod photoreceptor cells at P30
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• signal intensities of bipolar dendritic markers are reduced at P14
• synaptic ribbons and tips of bipolar dendrites are ectopically localized in the ONL at P21 and P30
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• cone bipolar cell dendrites abnormally extend together with horizontal processes into the ONL
|
• synaptic ribbons and tips of horizontal processes are ectopically localized in the ONL at P21 and P30
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• at P21, outer segments of both rod and cone photoreceptors are significantly shorter than wild-type
• however, the disk structure of outer segments remains normal
|
• outer segments of S- and M-cone photoreceptors are markdely shortened at both P14 and P30
• the length of M-cone photoreceptor outer segments is significantly shorter at P21
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• outer segments of rod photoreceptors are shortened at both P14 and P30
• the length of rod photoreceptor outer segments is significantly shorter at P21
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• progressive photoreceptor degeneration
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