Analysis Tools
vision/eye
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• severe retinal dysplasia, predominately affecting the photoreceptor layer
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• bipolar cell disorganization is seen at P10, with some cell bodies interspersed with photoreceptors, and becomes more severe at P21
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• apicobasal polarity of developing photoreceptors is disrupted as indicated by a failure of ciliary rootlets to align along the apical margin of the retina and marker analysis
• basal bodies are displaced throughout the developing neuroblast layer as early as P0
• majority of basal bodies lack a docked ciliary vesicle, indicating inhibited ciliogenesis
• dividing cells are frequently seen ectopically in the mid-neuroblast layer
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• separation of the inner nuclear layer and outer nuclear layer is disrupted at P5 and by P10, the outer nuclear layer is split and fragmented
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• disruption of the outer retina lamination is seen at P5 but not P0
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• ciliary rootlets are scattered throughout all retinal layers and the connecting cilium is absent at P5
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• inner segment is absent at maturity
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• outer segment is absent at maturity
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• ERG a- and b-waves are absent in both dark- and light-adapted mice at 6 weeks of age, indicating loss of both rod and cone function
• mice are non-responsive to a 10-Hz flicker test
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• amplitude of dark adapted a-wave is decreased
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• amplitudes of dark adapted and light adapted b-wave are abolished
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• b-waves are absent in both dark- and light-adapted mice
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nervous system
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• ciliary rootlets are scattered throughout all retinal layers and the connecting cilium is absent at P5
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• bipolar cell disorganization is seen at P10, with some cell bodies interspersed with photoreceptors, and becomes more severe at P21
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• inner segment is absent at maturity
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• outer segment is absent at maturity
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cellular
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• ciliary rootlets are scattered throughout all retinal layers and the connecting cilium is absent at P5
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