Analysis Tools
nervous system
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• at high magnification, but not evident at low magnification, approximately 5% of the neuromuscular junctions assesed in the levator auris longus at 4 months of age are found to be only partially innervated, far fewer than in Nmf249 heterozygotes
• nearly all terminals assessed in the levator auris longus show more diffuse postsynaptic staining, less distinct gutters, thinner axons and presynaptic nerves, synapses that are smaller than in wild-type controls, the average quantal content is significantly lower than normal, and the quantal content and mean evoked endplate currents are lower still at 4 months compared with 2 months
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• at 2 months of age 25% decrease in amplitude of evoked endplate currents, and quantal content in neuromuscular synapses of the levator auris longus muscle, and treatment with 3,4-DAP or physostigmine increases the in vivo evoked endplate currents at 4 months of age
• at 2 months of age the frequency of spontaneous release is consistently lower than normal although the quantal amplitude at synapses is normal, and no differences are found in the miniature endplate current amplitude
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behavior/neurological
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• compared with wild-type controls, heterozygotes have impaired grip strength and this is improved by treatment with physostigmine, but made worse by treatment with 3,4-DAP
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