mortality/aging
• some mice died between 18-30 days of age while others survived past 90 d of age
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behavior/neurological
• onset between 16-20 days of age; apparent at rest, but more pronounced during movement
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• mice exhibited jerking movement at 12-14 days of age
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• abnormal splaying and dragging of hindlimbs with clenched paws; progressive loss of hindlimb function leading to loss of locomotor ability by 60 d of age
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growth/size/body
• mice weighed 33-66% less than heterozygous and wild-type littermates
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homeostasis/metabolism
• absence of galactocerebroside and sulfatide
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immune system
N |
• normal natural killer T cell development and function
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nervous system
N |
• normal myelin ultrastructure; no vacuolar degeneration observed in PNS myelin
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• compact myelin displayed at 24 d of age
• decrease in thickness of dorsal spinal white matter myelin
• 30% decrease in mean thickness of ventral spinal white matter myelin
• myelin splitting evident in mice 24 d of age, progression with age
• vacuolization evident in the ventral region of the spinal cord in mice 24 d of age, progression with age
• abnormal node and paranode formation
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• associated with increased distribution of potassium channel, extending through internodal region
(J:58935)
• 50% reduction in compound action potential amplitude; 30-40% reduction in conductance velocity of sciatic nerve
(J:78426)
|