Analysis Tools
mortality/aging
|
• mutant pups are born at appropriate Mendelian ratio
• after birth, about one third of mutant mice die on postnatal day 1
• all remaining mutant mice die by P17
|
behavior/neurological
|
• mice surviving beyond P1 cannot easily regain posture when placed on their backs
|
|
• mice surviving beyond P1 display uncoordinated limb movements, and move primarily by writhing on their abdomen
|
growth/size/body
|
• mice surviving beyond P1 are severely runted, likely due to feeding problems secondary to their movement disorder
|
nervous system
|
• mitochondria in mutant Purkinje cells in mixed cerebellar cultures tend to cluster together in the cell body and do not enter the dendritic tracts
|
|
• markedly higher levels of apoptosis in mutant cerebella as early as p6 and continuing through the second postnatal week
|
|
• widespread loss of Purkinje cell bodies py P15 resulting in reduction of the molecular layer
• Purkinje cell loss due to a degenerative process in mixed cerebellar cultures
|
|
• reduced growth and deterioration of Purkinje cell dendrite during the second postnatal week
|
|
• severe defect in postnatal cerebellar growth
• between P7 and P16, the mutant cerebellum reduces in size
• lobular organization and formation of the three cellular layers is relatively intact
|
cellular
|
• mitochondria in mutant Purkinje cells in mixed cerebellar cultures tend to cluster together in the cell body and do not enter the dendritic tracts
|
|
• markedly higher levels of apoptosis in mutant cerebella as early as p6 and continuing through the second postnatal week
|
