Analysis Tools
behavior/neurological
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• 20 weeks of dox treatment results in a lower gain of optokinetic reflex, indicating deficits in compensatory eye movements
• discontinuing dox induction after 8 weeks of exposure results in better optokinetic reflex performance at 20 weeks
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• mice exhibit a lower gain in visually enhanced vestibulo-ocular reflex (VVOR) after 20 weeks of dox treatment
• discontinuing dox induction after 8 weeks of exposure prevents further deterioration of VVOR
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hearing/vestibular/ear
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• mice exhibit a lower gain in visually enhanced vestibulo-ocular reflex (VVOR) after 20 weeks of dox treatment
• discontinuing dox induction after 8 weeks of exposure prevents further deterioration of VVOR
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nervous system
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• mice exhibit ubiquitin-positive intranuclear inclusions in the brain (lobule X of the cerebellum, hippocampus and striatum) after 8 weeks of doxycycline (dox) treatment
• intranuclear inclusions are round/spherical in shape in the hippocampus and cat-eye shaped in the granular layer of lobule X of the cerebellum
• the number of inclusions and size of inclusions increase with longer dox treatment times
• ubiquitin-positive intranuclear inclusions contain polyglycine peptides, the 20S core complex of the proteasome, Hsp40, and Rad23B
• 12 weeks of dox washout after both 12 and 16 weeks of dox treatment does not reduce the number or size of ubiquitin-positive inclusions in cerebellar lobule X, however further increase in number and size is not seen, indicating prevention of further progression
• 12 weeks of dox washout after 8 weeks of dox treatment results in a reduction in the number and size of ubiquitin-positive inclusions
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| fragile X-associated tremor/ataxia syndrome | DOID:0050879 |
OMIM:300623 |
J:224760 | |
