Analysis Tools
nervous system
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• following middle cerebral artery occlusion and reperfusion, homozygotes exhibit smaller volumes of brain infarctions and lower brain edemas than wild-type controls (38 +/- 2% vs 60 +/- 6% and 40% vs 66%, respectively)
• in addition, mutant brain neocortex displays less severe ultrastructural changes and endothelial swelling in the ischemic core relative to wild-type brain
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• under basal conditions, homozygotes show a selective increase in the volume of perivascular astroglial end-feet in the neocortex (1.5-fold) and cerebellum (1.7-fold) relative to wild-type controls, consistent with loss of AQP4 from perivascular and subpial membranes but not from other membrane domains
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• mutant sternomastoid neuromuscular junctions (NMJs) display shallow nerve gutters, postjunctional folds that appear less organized and have fewer openings to the synaptic cleft than controls, synaptic acetylcholine receptors (AChRs) separated into distinct clusters, and perisynaptic clusters of AChR extending beyond identifiable nerve-muscle contacts
• mutant neuromuscular junctions have undetectable levels of postsynaptic utrophin and show a significant reduction in AChR (65%) and acetylcholinesterase (55%) levels relative to wild-type controls
• some NMJs display these features over their entire extent, whereas others show areas of aberrant AChR pattern adjacent to normally appearing areas
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homeostasis/metabolism
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• in the basal state, loss of AQP4 at the brain-blood interface appears to impede the efflux of water generated from brain energy metabolism and results in swollen perivascular astroglial end-feet in mutant brains
• selective depletion of the perivascular AQP4 pool reduces the volume of postischemic brain edema, putatively due to reduced water influx
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• neuronal nitric oxide synthase 1 (NOS1), a component of the dystrophin protein complex, is nearly absent from the sarcolemma, even where other syntrophin isoforms are present
• however, homozygotes are able to exercise and run for similar times and distances as wild-type controls in the voluntary exercise test
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• following middle cerebral artery occlusion and reperfusion, homozygotes exhibit smaller volumes of brain infarctions and lower brain edemas than wild-type controls (38 +/- 2% vs 60 +/- 6% and 40% vs 66%, respectively)
• in addition, mutant brain neocortex displays less severe ultrastructural changes and endothelial swelling in the ischemic core relative to wild-type brain
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muscle
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• homozygotes are mobile (as shown by voluntary running wheel experiments) and display no overt signs of muscular dystrophy
• skeletal muscle appears histologically normal, with minimal fibrosis, few centralized nuclei, and a normal size distribution of muscle fibers
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