Analysis Tools
nervous system
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• at E18.5, a 20% decrease in motor neuron nuclei is detected, with a 42% reduction in adult animals
• no further loss of motor neurons is observed after axotomy compared to wild-type
• no increase in cell death after nerve lesion is observed relative to controls
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• at E18.5, a 22% decrease in motor neuron nuclei is detected, with a 37% decrease observed in adult mice
• after axotomy, almost 2x the number of regenerating motor neurons are lost compared to wild type animals
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• synaptic maturation is impaired; quantification of synaptophysin, synapsin and AChR shows loss of synaptophysin and synapsin at E18 but not in adult mice, but AChR quantification is similar between genotypes
• adult mice have significantly fewer endplates than control mice
• after transection of the hypoglossal nerve, size of the AChR postsynaptic membrane is larger in the reinnervated synapses in lesioned mutants with more diffuse AChR localizations within the specialization; numbers of neurofilament-positive fibers and volume occupied by these fibers are not different from control mice
• reduction of AChRs, synapsin I and synaptophysin is observed compared to lesioned controls, but number os endplates is similar between lesioned control and mutant animals
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renal/urinary system
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• kidneys develop normally
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