Analysis Tools
mortality/aging
|
• at 6 months of age mice do not show overt signs of disease but deteriorate rapidly after this
• average life span of 373 to 404 days
• when given doxycycline starting at 5 weeks of age, average life span is 172.5 days
|
nervous system
microgliosis
(
J:190450
)
|
• activation of microglia accompanies glycoconjugate storage
|
|
• glycoconjugate storage is seen in the many areas of the brain but not in the cerebral cortex or most other cerebral structures
• substantial glycoconjugate storage is seen in the hindbrain and ventral spinal cord by one year of age
• glycoconjugate storage is increased following doxycycline treatment
|
|
• decrease in the density of interneurons in the ventral horn
|
behavior/neurological
|
• when given doxycycline starting at 5 weeks of age, a dramatic decrease in motor performance is seen starting at 20 weeks of age
|
|
• progressive tremor develops after 6 months of age
• tremors are milder than in mutant mice not expressing the transgene
• develop progressive tremor from 17-19 weeks of age when given doxycycline starting at 5 weeks of age
|
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• hindlimbs appear uncoordinated when walking
|
bradykinesia
(
J:190450
)
|
• seen when mice are at their humane endpoint, when given doxycycline starting at 5 weeks of age
|
muscle
|
• progressive limb hypertonia after 6 months of age
• by 1 year of age limb hypertonicity restricts movement
|
growth/size/body
weight loss
(
J:190450
)
|
• when given doxycycline starting at 5 weeks of age, weight plateaus at 20 weeks of age and then starts to decrease
|
hematopoietic system
microgliosis
(
J:190450
)
|
• activation of microglia accompanies glycoconjugate storage
|
immune system
microgliosis
(
J:190450
)
|
• activation of microglia accompanies glycoconjugate storage
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Sandhoff disease | DOID:3323 |
OMIM:268800 |
J:190450 | |
