Analysis Tools
mortality/aging
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• most die around weaning; however, with special nursing most homozygotes can survive to adulthood
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nervous system
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• the inferior olivary nucleus is 19% and 20% shorter than wild-type at 53 and 150 days of age
• about a 12% reduction in cell number is also seen at 53 and 150 days of age
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• nuclei diameters are slightly smaller
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• Purkinje cells have an atrophic dendritic tree, proximal segments that are longer with fewer branches, distal spiny branchlets emerging from primary dendrites, and proximal dendrites studded with spines
• Purkinje cell axons have abnormal dendritic-like processes interposed between the cell body and the initial segment of the axon, axonal enlargements in the myelinated segments and some axon terminals undergo a slow degenerative process
• spines arising from the thick dendritic segments are abnormally persistent somatic filopodia
• however, synaptic connections to parallel fibers appear normal, little Purkinje cell death is seen, and excitatory postsynaptic potentials are similar to wild-type
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• about a 27% decrease in Purkinje cell numbers that is regularly distributed along the mediolateral axis
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• the cerebellar cortex is reduced in size by about 20%; however foliation and lamination are normal
(J:7047)
• cerebellar cortex is smaller and the cerebellar laterolateral extent is reduced by 11.5%
(J:106373)
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behavior/neurological
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• unable to stand after being placed on their back
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• by 6 - 8 days of age homozygotes begin to lean to one side and develop difficulty in coordinating movements
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• no vaginal plugs are found
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reproductive system
growth/size/body
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• slow growth from P8-10 onward
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