mortality/aging
|
• none survive past P8
|
muscle
|
• skeletal muscle myofibers lack well-defined A- and I- bands with areas of normal-looking sarcomeres unlike in wild-type mice
|
|
• accumulation of F-actin
|
|
• skeletal muscle myofibers lack well-defined A- and I- bands with areas of normal-looking sarcomeres unlike in wild-type mice
• skeletal muscle myofibers accumulate abnormal mitochondria unlike in wild-type mice
|
|
• occasionally at P3
• extensive and severe at P7
• at P7, mice exhibit ballooning degeneration interspersed with normal-looking myofibers and pale core-like areas consistent with myofibrillar disruption and absent mitochondria unlike in wild-type mice
|
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• increase in the mean percentage of slow fibers
|
|
• mice exhibit muscle weakness
• however, mice exhibit normal neuromuscular junctions and nerve myelination
|
growth/size/body
|
• by P7
|
|
• by P7
|
|
• from P3 and P7
|
behavior/neurological
|
• little to no mild in the stomach suggests inability to suckle by P7
|
|
• little to no mild in the stomach suggests inability to suckle by P7
|
|
• reduced spontaneous activity
|
nervous system
| N |
• mice exhibit normal neuromuscular junctions and nerve myelination
|


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