mortality/aging
• 100% die at some point after 12 months but before a normal life span
|
behavior/neurological
N |
• no abnormal phenotype in young mice
• robust righting response
|
• clasp hind limbs when lifted by the tail
|
• high frequency nodding tremors which are absent in resting mice
• develops as early as four months but usually at 6-8 months in males
• tremors develop at 10-12 months in females
|
• hind limb weakness followed by forelimb weakness
• earlier onset of weakness in males than in females
• eventually unable to support their heads
|
• develop a spastic gait
|
growth/size/body
weight loss
(
J:150794
)
• weight loss and weakness starting around 10-12 months
• less severe when on a wet diet
|
nervous system
• in affected animals
• but never by more than 25%
|
astrocytosis
(
J:150794
)
• in the granular layer
|
• defects in both ascending and descending tracts of the spinal cord
• massive axonal degeneration and absence of largest caliber axons
• swollen axons
• membrane derived axonal inclusions and glycogen inclusions
• nodal gaps are sometimes widened
|
reproductive system
• females are sub-fertile
|
renal/urinary system
homeostasis/metabolism
• moderately elevated
|