mortality/aging
• in few cases, mice of this genotype survived up to 6 days of age
|
• the majority of mice were stillborn or died within 6 hours of birth
• a slightly less than expected frequency of mice with this genotype suggests that some mice may be dying in utero
• in few cases, mice of this genotype survived up to 6 days of age
|
behavior/neurological
• mice that survived 4-6 days displayed a decrease or lack of suckling by 48 hours postnatal
|
• mice that survived 4-6 days showed a marked tremor within 72-96 hours postnatal
|
bradykinesia
(
J:60592
)
• mice that survived 4-6 days showed a decrease in movement within 48-72 hours postnatal
|
muscle
N |
• no musculature atrophy was detected in the quadriceps or gastrocnemius of mice that survived 4-6 days
|
respiratory system
• mice that survived 4-6 days displayed labored breathing by 48 hours postnatal
|
nervous system
• dramatic loss of motor neurons were observed in spinal cord (~35% loss) and facial nucleus(~40% loss) at postnatal day 5
• normal numbers of motor neurons were observed in spinal cord and facial nucleus in animals at postnatal day 1
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Werdnig-Hoffmann disease | DOID:13137 |
OMIM:253300 |
J:60592 |