growth/size/body
weight loss
(
J:96574
)
• body weight progressively decreases, with weight only 40-60% of controls at moribund stage
|
behavior/neurological
• excessive grooming is observed at 2-4 months
|
• progressively deteriorating from 2.5 months
|
limb grasping
(
J:96574
)
• at 2-4 months, mice display transiently clutched paws; at 4-8 months, paws are permanently clenched
|
• at 2-4 months, mice exhibit lateral body displacement while climbing, and display transiently uncoordinated limb extension (much more prolonged at 4-8 months)
|
• mice can not remain on rotating rod, starting at 1.75 months
|
• grip strength in fore- and hindlimbs is progressively impaired from 1.75 months of age
|
abnormal gait
(
J:96574
)
• mice develop progressively deteriorating gait from 2-4 months of age (gait is wide-based initially, wide-based and dragged at late stage, and scribbled at later to moribund stages)
|
• at 2-4 months, mice exhibit lateral body displacement while climbing, and display transiently uncoordinated limb extension (much more prolonged at 4-8 months)
|
nervous system
• these are prominent (larger and more abundant) in neurons in selected brain regions and spinal cord compared to wild-type controls a 3-4 months worsening with age; in spinal cord, inclusions are observed in motor and sensory neurons
|
reproductive system
azoospermia
(
J:121039
)
|
skeleton
• starts to develop at 2-4 months; at 4-8 months, mice show pronounced hunchback with low pelvic elevation and muscle wasting
|
homeostasis/metabolism
• serum concentration is significantly lower in males than in wild-type males
|
• serum concentration is significantly lower in males than in wild-type males
|
• significantly lower in transgenic females compared to wild-type females
|
• significantly lower than in wild-type males
|
endocrine/exocrine glands
cellular
azoospermia
(
J:121039
)
|