mortality/aging
• 5 of 37 die within 6 months
|
behavior/neurological
• difficulties in hindlimb movement
|
abnormal gait
(
J:75421
)
• gait abnormalities
|
growth/size/body
limbs/digits/tail
kinked tail
(
J:75421
)
• kinked or serpentine tails
|
nervous system
• at P3.5, see a 10-fold reduction in ventral and a 5-fold reduction in dorsal roots, with this reduction still apparent at P15, however by 6 months of age, cell numbers are similar to those in controls, however myelin sheaths formed are thin
|
• reduction in thickness of the myelin sheath of sciatic nerves at P15 that persists at 6 and 14 months of age
• thin myelin is due to the presence of fewer wraps of myelin around the axon
|
• dorsal roots display a severe decrease of Schwann cells in all mutants at P3.5 and P15
|
• sciatic nerves at P15 are translucent and thin
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• ventral roots display a severe decrease of Schwann cells in all mutants at P3.5 and P15
• about 20% loss of axons in the L5 ventral root
|
• hypomyelination in sciatic, sural, saphenous nerves, nerves innervating lower leg muscles, and the dorsal spinal roots and absent myelin in ventral spinal roots
• occasionally see large-caliber axons lacking myelin that are still surrounded by a Schwann cell basal lamina in the sciatic nerve
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