mortality/aging
• mice are sacrificed at 5 to 6 weeks of age due to deteriorating condition
|
nervous system
• axon degeneration is followed by secondary degeneration of myelin sheaths
|
• at 4 weeks of age, mice exhibit axonal degeneration that results in the loss of myelinated axons in the skin dermis
• axonal degeneration is observed in the sciatic nerve and peripheral nerves
• axon degeneration is followed by secondary degeneration of myelin sheaths
• degenerative changes occur in the gray matter of the brain and spinal cord
|
muscle
• at 1 month of age, mice exhibit hind limb atrophy unlike in wild-type mice
|
behavior/neurological
homeostasis/metabolism
• mice exhibit delayed wound healing due to decreased keratinocyte migration into the wound compared to in wild-type mice
|
limbs/digits/tail
kinked tail
(
J:24974
)
• at 1 month of age, mice exhibit kinked and rigid tails unlike in wild-type mice
|
integument
• older mice exhibit hair loss in tuffs following gentle pulling unlike in wild-type mice
|
blistering
(
J:24974
)
• unlike in wild-type mice, tail skin blisters as a result of mechanical stress
• blistering occurs from basal cells rupturing in a highly defined zone parallel to and just above cell base
• blistering in stratified squamous epithelia and in epidermis of haired skin is rare
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