Analysis Tools
mortality/aging
| N |
• double mutants are born at the expected Mendelian ratio when mothers are treated with doxycycline to suppress H2-K1 expression
|
|
• in the absence of doxycycline, no pups are seen, although mutants are identified among live 18-day embryos and several mummified mutant fetuses are retained in the uterus, suggesting parturition problems
|
growth/size/body
 |
• females in which doxycycline is removed at 4 weeks of age show a 40-50% reduction in body weight at 6 months of age
|
|
|
• females in which doxycycline is removed at 4 weeks of age show arched spine, lower back and hind limb weakness and wasting becomes severe by 5-7 months of age
|
behavior/neurological
|
|
• females in which doxycycline is removed at 4 weeks of age show a reduction in locomotor activity in an open field as early as 1 month after doxycycline removal and is striking by 5-7 months of age
|
muscle
|
• females in which doxycycline is removed at 4 weeks of age show macrophage infiltration in skeletal muscle by 3.5 months of age and mononuclear cell infiltration and invasion of some muscle fibers by phagocytes by 5.5 months of age
• males remain free of symptoms up to 5 months of age, but they all develop symptoms after 7 months of age
• administration of doxycycline at 4 months of age after the establishment of clinical disease for 5 months, fails to reverse the disease
|
|
|
• females in which doxycycline is removed at 4 weeks of age exhibit abnormal skeletal muscles with centralized nuclei, muscle fiber degeneration, and macrophage infiltration by 3.5 months of age and by 5.5 months, there is structural damage, showing centralized nuclei, variation in muscle fiber diameter, muscle fiber degeneration and regeneration, atrophy, obliteration of the striations of the contractile apparatus, mononuclear cell infiltration, and the invasion of some muscle fibers by phagocytes
|
|
|
• females in which doxycycline is removed at 4 weeks of age show muscle fiber atrophy by 5.5 months of age
|
|
|
• females in which doxycycline is removed at 4 weeks of age show muscle fiber degeneration by 3.5 months of age
|
|
|
• females develop signs of muscle weakness at about 3 months of age (2 months after transgene induction by doxycycline removal)
|
immune system
|
|
• MIP1-alpha, MCP-1, and IL-15 are increased in muscle tissues of females in which doxycycline is removed at 4 weeks of age
|
|
|
• 8 of 23 mutants females in which doxycycline is removed at 4 weeks of age show elevated autoantibodies to histidyl-tRNA synthetase
|
|
|
• 5 of 18 females in which doxycycline is removed at 4 weeks of age show antinuclear antibodies
|
|
• females in which doxycycline is removed at 4 weeks of age show macrophage infiltration in skeletal muscle by 3.5 months of age and mononuclear cell infiltration and invasion of some muscle fibers by phagocytes by 5.5 months of age
• males remain free of symptoms up to 5 months of age, but they all develop symptoms after 7 months of age
• administration of doxycycline at 4 months of age after the establishment of clinical disease for 5 months, fails to reverse the disease
|
homeostasis/metabolism
|
|
• females in which doxycycline is removed at 4 weeks of age show an increase in serum levels of glutamic-oxaloacetic transaminase, indicating muscle parenchymal damage
|
|
|
• females in which doxycycline is removed at 4 weeks of age show an increase in serum levels of creatine kinase, indicating muscle parenchymal damage
|
|
|
• MIP1-alpha, MCP-1, and IL-15 are increased in muscle tissues of females in which doxycycline is removed at 4 weeks of age
|
skeleton
|
|
• females in which doxycycline is removed at 4 weeks of age, show arched spine by 5-7 months of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| myositis | DOID:633 |
OMIM:160750 |
J:205907 | |
