Analysis Tools
behavior/neurological
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• 58% of mice exhibit decreased splay angles of hindlimb limbs when lifted by the tail compared to 33% of wild-type mice, with earlier onset of 80 days compared to 140 days of age in wild-type mice
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• 100% of mice exhibit resting tremor at 247 days of age compared to 6.7% of wild-type mice, with onset at 83 days of age
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• mice exhibit deficits in gripping the beam in the beam walk assay indicating impaired gait
• females show less severe motor defects than males
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growth/size/body
weight loss
(
J:273408
)
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• a decrease in weight is seen in mice older than P150
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nervous system
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• dorsal and ventral columns show decreased numbers of regular axons and increased numbers of irregular axons, indicating axonal dieback and corticospinal degeneration
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• dorsal and ventral columns show decreased numbers of regular axons and increased numbers of irregular axons, indicating axonal dieback
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• spinal cord, particularly the lower spinal cord, exhibits a reduction in microtubule stability
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• progressive axonal degeneration in the dorsal column
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• cortical neurons from P0 mice show a decrease in retrograde movements of axonal lysosomes, with an increase in movements that are halted or stationary
• however, no instantaneous velocity differences in lysosomal movement are seen
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| hereditary spastic paraplegia 4 | DOID:0110792 |
OMIM:182601 |
J:273408 | |
