Analysis Tools
behavior/neurological
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• Background Sensitivity: clinical signs persist from 5-6 weeks to more than a year of age
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hyperactivity
(
J:14906
)
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• Background Sensitivity: severity varies with genetic background
• behavior displays by 3.5 weeks of age and becomes more pronounced with time
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growth/size/body
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• size differential is recognizeable by two weeks of age
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cellular
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• a preliminary EM study shows mitochondria of photoreceptor inner segments are larger than normal
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• at 9 days after birth rounded mitochiondria are seen in perikarya of some Purkinje cells
• by 15 days all Purkinje cells contain spherical mitochondria
• most mitochondria degenerate but some Purkinje cells reaquire normal-appearing mitochondria
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reproductive system
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• the abnormal neurological behavior renders both males and females poor breeders
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nervous system
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• at 9 days after birth rounded mitochiondria are seen in perikarya of some Purkinje cells
• by 15 days all Purkinje cells contain spherical mitochondria
• most mitochondria degenerate but some Purkinje cells reaquire normal-appearing mitochondria
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• between 23 and 50 days of age there is a 90% loss of cerebellar Purkinje cells
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• mitochondria of photoreceptor inner segments are larger than normal
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• large whorls of outer segment membranes seen as early as 3 weeks of age accumulate so that by 2.5-3 months of age the thickened outer segment zone is comprised exclusively of whorls with distinct outer segments
• with progressive loss of photoreceptors the outer segment zone becomes thinner after 3-4 months of age until no outer segment membranes are seen at 11 months of age
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• slightly shorter than those of littermate controls
• conspicuous in mice 19 days of age
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• conspicuous in mice 19 days of age
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• slow, progressive degeneration
• 20%-30% of cells disappear by 3 weeks of age
• loss parallels development of cerebellar ataxia
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vision/eye
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• first evident at 2-3 weeks of age
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• mitochondria of photoreceptor inner segments are larger than normal
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• large whorls of outer segment membranes seen as early as 3 weeks of age accumulate so that by 2.5-3 months of age the thickened outer segment zone is comprised exclusively of whorls with distinct outer segments
• with progressive loss of photoreceptors the outer segment zone becomes thinner after 3-4 months of age until no outer segment membranes are seen at 11 months of age
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• slightly shorter than those of littermate controls
• conspicuous in mice 19 days of age
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• conspicuous in mice 19 days of age
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• slow, progressive degeneration
• 20%-30% of cells disappear by 3 weeks of age
• loss parallels development of cerebellar ataxia
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