Analysis Tools
behavior/neurological
|
• mice show marked ataxia at 3 and 6 months of age
• some mice show poor performance on the balance beam test and cannot cross the beam because they fall down or are unable to perform the task
|
|
• 3- and 6-month old mice show decreased latency to fall off the accelerating rotarod
|
|
• footprint gait analysis of 9-month-old mice shows an ataxic gait with increased step width
|
nervous system
|
• dendritic expansion of Purkinje cells in organotypic slice culture is severely impaired and cells develop only short thickened dendrites
• cerebellar slice cultures treated with PKC inhibitor Go6983 have longer dendrites with more branches than untreated, showing a significant rescue
• Purkinje cells show less extended, condensed dendritic trees covering a reduced area in 4-week-old and 1-year-old mice
• however, no obvious Purkinje cell loss is seen from 4 to 40 weeks of age
|
|
• the thickness of the molecular layer in lobule VII is slightly reduced in 40-week-old mice
|
|
• climbing fiber terminals have reduced density, in particular, on the distal part of the dendrites
• 40-week-old mice show significantly less climbing fiber terminals in the molecular layer on the distal part of the dendrites, indicating disturbed climbing fiber innervation
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| spinocerebellar ataxia type 14 | DOID:0050964 |
OMIM:605361 |
J:305637 | |
