mortality/aging
• severely affected mice are euthanized
|
muscle
• increased numbers of atrophic type I fibers
|
• severity depends on affected muscle
|
• muscle wasting in posterior legs by 6 to 8 weeks
• widespread and conspicuous neurogenic atrophy in skeletal muscles with severity depending on affected muscle
|
nervous system
microgliosis
(
J:192445
)
• mild to moderate
|
astrocytosis
(
J:192445
)
• mild to moderate
|
• cytoplasmic inclusions
|
• fewer myelinated axons at 8 weeks especially large caliber axons
|
behavior/neurological
• progressive motor dysfunction
• at 4 weeks, mice spend less time hanging from an inverted cage grid than wild-type mice
|
• at 4 weeks, mice spend less time hanging from an inverted cage grid than wild-type mice
|
• posterior waddle by 4 weeks
|
• by 4 weeks
|
• at 8 to 20 weeks
|
skeleton
• at 8 to 20 weeks, mice exhibit dorsoventral flattening of the pelvis
|
immune system
microgliosis
(
J:192445
)
• mild to moderate
|
growth/size/body
hematopoietic system
microgliosis
(
J:192445
)
• mild to moderate
|