vision/eye
• extracellular vesicles (ectosomes) with uniform diameter are seen in the subretinal space at P10
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• marker analysis indicates that axonemal/ciliary extension is disrupted in the retina
• photoreceptors show shorter ciliary rootlets at P10
• basal bodies are displaced and often seen within the inner segments adjacent to the outer limiting membrane indicating that basal bodies do not dock properly at the apical edge of the inner segments
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• shorter photoreceptor inner segments at P10
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• reduction of photoreceptor outer segment-resident proteins at P10
• P10 retinas show a complete absence of recognizable outer segments with a few outer segment rudiments that are highly vesiculated and lack the stacked discs
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• photoreceptors fail to develop outer segment membranous discs
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• photoreceptor cell death is seen at P10 and by P27, rapid photoreceptor degeneration with extensive loss is seen in the central retina compared with the peripheral edges
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• initial development of photoreceptors is defective as indicated by a 58% and 71.4% decrease in the number of pHH3+ and Ki-67+ (proliferating) retinal progenitor cells, respectively, and a decrease in apoptosis in P5 retina
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• retinal thickness is reduced at P5
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• ERG analysis indicates a severe loss of rod (scotopic) and cone (photopic) photoreceptor function
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nervous system
• marker analysis indicates that axonemal/ciliary extension is disrupted in the retina
• photoreceptors show shorter ciliary rootlets at P10
• basal bodies are displaced and often seen within the inner segments adjacent to the outer limiting membrane indicating that basal bodies do not dock properly at the apical edge of the inner segments
|
• shorter photoreceptor inner segments at P10
|
• reduction of photoreceptor outer segment-resident proteins at P10
• P10 retinas show a complete absence of recognizable outer segments with a few outer segment rudiments that are highly vesiculated and lack the stacked discs
|
• photoreceptors fail to develop outer segment membranous discs
|
• photoreceptor cell death is seen at P10 and by P27, rapid photoreceptor degeneration with extensive loss is seen in the central retina compared with the peripheral edges
|
cellular
• marker analysis indicates that axonemal/ciliary extension is disrupted in the retina
• photoreceptors show shorter ciliary rootlets at P10
• basal bodies are displaced and often seen within the inner segments adjacent to the outer limiting membrane indicating that basal bodies do not dock properly at the apical edge of the inner segments
|