cellular
• mitochondria in Purkinje cells are enlarged compared to in wild-type cells
|
mortality/aging
• mice die within 36 hours of birth
|
nervous system
• at P0, cerebellum are smooth and exhibit a 60% decreased in size compared to in wild-type mice
|
• at P0, cerebellum lack foliation
• at P0.5, lobule fissures are barely detectable
|
• Purkinje cells in the cerebellum are decreased in number and form a discontinuous cell layer unlike in wild-type mice
|
• mitochondria in Purkinje cells are enlarged compared to in wild-type cells
|
• in the cerebellum
|
• at P0, cerebellum are 60% smaller than in wild-type mice due to decreased cell proliferation
|
behavior/neurological
• mice die without milk in their stomach
• however, mice exhibit normal swallowing when manually fed milk
|