mortality/aging
• pups are born alive, but animals die shortly after birth
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behavior/neurological
limp posture
(
J:60159
)
• prominent head and forelimb drop was observed
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homeostasis/metabolism
muscle
• abnormal distribution of nuclei; appeared centrally instead of peripherally
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• decrease in mass and a 50% decrease in muscle fiber number
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• regions of immature-appearing muscle were observed
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respiratory system
• lung alveloi failed to expand, demonstrating that these animals never inspired
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nervous system
• progressive loss through development
• absent from peripheral nerve terminals
• present close to dorsal and ventral roots
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• progressive withdrawal of motor neuron projections after E14.5
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• defasciculated and extensively branched
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• 60% reduction in somatic motor neurons seen at E18.5
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• 60% reduction in Islet1+ cells in dorsal root ganglia
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• cranial ganglia were reduced in size
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• cranial nerves were reduced in size
• terminal extensions of these nerves were abnormally branched and defasciculated, or in some cases, absent
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• reduced size at E18.5
• the number of TrkA+ cells in lumbar dorsal root ganglia was reduced at E18.5 but not at E14.5
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• the intercostal and lumbar nerves showed defects in fasciculation, branching and in the nerve terminals
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• the phrenic nerve was absent at P0; at E14.5, the nerve was present but branched abnormally and nerve terminals in the diaphragm did not localize to the endplate zone
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• at E14.5, phrenic nerves appeared desfaciculated and had aberrant branching
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cellular
• at E14.5, phrenic nerves appeared desfaciculated and had aberrant branching
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