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 2-3 months
|
• progressively deteriorating from 2.5 months
|
limb grasping
(
J:96574
)
• at 2-3 months, mice display transiently clutched paws; at 3-5 months, paws are permanently clenched
|
• at 2-3 months, mice exhibit lateral body displacement while climbing, and display transiently uncoordinated limb extension (much more prolonged at 3-5 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 2-3 months of age (gait is wide-based initially, wide-based and dragged at late stage, and scribbled at later to moribund stages)
|
• open-field activity is lower and progressively decreases from 1.75 months
|
nervous system
• total number of tyrosine hydroxylase-positive neurons in substantia nigra is 38% lower than in controls
|
• 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
homeostasis/metabolism
• serum concentration is significantly lower in males than in wild-type males
|
skeleton
• starts to develop at 2-3months; at 3-5 months, mice show pronounced hunchback with low pelvic elevation and muscle wasting
|
endocrine/exocrine glands