nervous system
N |
• at E12.5, embryos show normal numbers of motor neuron (MN) progenitors (Olig2+), generic MNs (Isl1/2+), and MN subtypes (Lhx3+ and Foxp1+) in the spinal cord relative to wild-type controls
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• Sholl analysis of primary motor neurons (MNs) shows a delay in the timing of neurite growth relative to wild-type MNs
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• on day 3 of in vitro culture (DIV-3), primary motor neurons (MNs) harvested from the spinal cord show significantly fewer intersections at ~20-120 um from the cell body, indicating reduced dendritic complexity
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• on day 3 of in vitro culture (DIV-3), primary motor neurons (MNs) harvested from the spinal cord show reduced dendritic complexity relative to wild-type MNs
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behavior/neurological
N |
• at 1.5 months of age, mice exhibit normal exploratory behavior (total distance traveled over 60 min) in the open field test
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• at 1.5 months of age, mice exhibit a significant decrease in the latency-to-fall (sec) in a rotarod test
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• at 1.5 months of age, mice exhibit significantly reduced forelimb grip strength relative to wild-type controls
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muscle
• adult mice exhibit muscle weakness
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cellular
• Sholl analysis of primary motor neurons (MNs) shows a delay in the timing of neurite growth relative to wild-type MNs
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mortality/aging
N |
• mice are born in expected Mendelian ratios, are viable after birth, and exhibit a normal lifespan relative to wild-type controls
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growth/size/body
N |
• at 1.5 months of age, mice appear grossly normal by X-ray micro-CT scanning and show normal body weight relative to wild-type controls
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