mortality/aging
• 57.6% of males and 84.4% of females survive longer (mean of 80-83 days) and die with whole body paralysis
• 17.4% of males, and no females, have a short lifespan and die with hindlimb paralysis at a mean of 38.9 days
• 25% of males and 15.6% of females show sudden death without paralysis with a mean of 33.6 days and 41.3 days in females and males, respectively
|
behavior/neurological
• discoordinated movements
|
head bobbing
(
J:207750
)
• unsteady gait
|
• mice show reduced vertical behaviors by 5 weeks of age
|
• after 10 weeks of age, mice are less active as they become progressively weaker
|
• mice exhibit hyperactivity beginning at 4 weeks of age, which peaks at around 5 weeks of age and ends around 10 weeks of age
|
• mice surviving the longest develop whole-body paralysis; this is seen in both males and females
|
• 17.4% of males, and no females, have a short lifespan and die with hindlimb paralysis
|
cardiovascular system
growth/size/body
weight loss
(
J:207750
)
• mice start to lose weight around 5 weeks of age
|
hematopoietic system
microgliosis
(
J:207750
)
• abundant microgliosis in the ventral horn gray matter
|
immune system
microgliosis
(
J:207750
)
• abundant microgliosis in the ventral horn gray matter
|
muscle
• necrotic skeletal muscle fibers and fibers with centralized nuclei in some areas of the muscle
|
• necrotic skeletal muscle fibers and fibers with centralized nuclei in some areas of the muscle
|
• electromyography indicates that mice develop progressive active denervation of muscles as indicated by the presence of positive sharp waves
|
nervous system
microgliosis
(
J:207750
)
• abundant microgliosis in the ventral horn gray matter
|
astrocytosis
(
J:207750
)
• astrogliosis in the deep layer of the frontal cortex
• abundant astrogliosis in the ventral horn gray matter
|
• neurodegeneration in layer V cortex and ventral horn spinal cord
|
• dramatic motor neuron loss, with about 60% fewer motor neurons in the ventral horn
|
• degeneration of axons in the ventral but not dorsal roots
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
amyotrophic lateral sclerosis type 10 | DOID:0060201 |
OMIM:612069 |
J:207750 |