mortality/aging
• mice exhibit a 50% survival rate after weaning
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• about 50% of homozygotes die within a week of birth
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immune system
• mice exhibit an increased frequency of IgA-expressing germinal center B cells in Peyers patches and a variable but, on average, twofold reduction in IgG1-expressing B cells, indicating an enhanced isotype switch to IgA production
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• mixed bone marrow chimera experiments indicate reduced numbers of germinal center B cells
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hematopoietic system
• mice exhibit an increased frequency of IgA-expressing germinal center B cells in Peyers patches and a variable but, on average, twofold reduction in IgG1-expressing B cells, indicating an enhanced isotype switch to IgA production
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• mixed bone marrow chimera experiments indicate reduced numbers of germinal center B cells
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nervous system
N |
• mice exhibit normal hippocampus morphology at 9-10 months of age
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• size of the readily releasable pool (RRP) of SVs is smaller than that in wild-type controls
• SV density is decreased in excitatory hippocampal neurons, indicating that neurons with only nCLCb clathrin cannot compensate for defective SV regeneration and show reduced SV pools from impaired replenishment
• other parameters, including individual SV size, postsynaptic density length and excitatory synapse density are similar to those in wild-type controls
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• in hippocampal neurons, the rate of synaptic vesicle (SV) recycling is decreased, indicating a defect in acute SV pool replenishment
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• basal synaptic transmission is impaired
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• in hippocampal slices, the evoked excitatory postsynaptic current (EPSC) response amplitude is reduced relative to that in wild-type controls
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• in hippocampal slices, the paired-pulse ratio (PPR) of evoked EPSCs at CA1 synapses is increased, consistent with a reduction in the probability of neurotransmitter release
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behavior/neurological
• surviving mice show defects in a grid-walking test
• however, grip strength is normal
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• surviving mice exhibit impaired motor coordination in an accelerating rotarod test
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