mortality/aging
• none survive past P8
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muscle
• skeletal muscle myofibers lack well-defined A- and I- bands with areas of normal-looking sarcomeres unlike in wild-type mice
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• accumulation of F-actin
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• 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
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• 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
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• mice exhibit muscle weakness
• however, mice exhibit normal neuromuscular junctions and nerve myelination
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growth/size/body
• by P7
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• by P7
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• from P3 and P7
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behavior/neurological
• little to no mild in the stomach suggests inability to suckle by P7
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• little to no mild in the stomach suggests inability to suckle by P7
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• reduced spontaneous activity
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nervous system
N |
• mice exhibit normal neuromuscular junctions and nerve myelination
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