mortality/aging
• usually between 4-6 months of age; variable and incomplete penetrance
|
behavior/neurological
• uncoordinated movements and frequent falls; variable age of onset, but seen as early as P16
|
abnormal gait
(
J:51377
)
• occasionally preceding death
|
nervous system
• occasionally preceding death
|
• reduced by 60% in number by 5 months of age; progressive reduction with age
|
• reduced in thickness, probably due to loss of Purkinje cells
|
• seen in mice greater than 5 months of age
|
• higher threshold required to initiate action potentials; occasional failure to fire action potential on stimulus and slower rate of maximal firing on long current stimulus
|