Mouse Genome Informatics
tg
    Tg(Lgi1*)#Mpan/0
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• mutants exhibit increased susceptibility to pentylenetetrazol-induced seizures, with mutants developing clonic seizures sooner than controls; however, once mutants are fully kindled, the latency and duration of seizures does not differ from controls, indicating that mutants are prone to kindling epileptogenesis
• however, EEG recording failed to show spontaneous seizures in mutants, even though excitatory transmission is increased, inhibitory transmission exceeds excitatory transmission by about 15-fold

nervous system
• mutants exhibit increased susceptibility to pentylenetetrazol-induced seizures, with mutants developing clonic seizures sooner than controls; however, once mutants are fully kindled, the latency and duration of seizures does not differ from controls, indicating that mutants are prone to kindling epileptogenesis
• however, EEG recording failed to show spontaneous seizures in mutants, even though excitatory transmission is increased, inhibitory transmission exceeds excitatory transmission by about 15-fold
• mature granule cells show a persistently wide and heavily branched apical dendritic arbor typical of immature wild-type granule cells, indicating an inhibition of postnatal pruning of granule cell apical dendrites in mutants
• mature granule cells show a persistently wide and heavily branched apical dendritic arbor typical of immature wild-type granule cells, indicating an inhibition of postnatal pruning of granule cell apical dendrites in mutants
• granule cells of adults however are not immature neurons as the maturation of their passive and active membrane properties occurs normally
• mutants show an arrest in development of hippocampal medial perforant path-granule cell excitatory synapses, causing an increase of excitatory synaptic transmission
• the decrease in hippocampal medial perforant path (MPP) release probability (presynaptic function) with maturation that normally happens in wild-type mice is blocked in mutants
• mutants exhibit an increase in excitatory synaptic transmission
• amplitude of AMPA receptor-mediated and NMDA receptor-mediated EPSCs, and the frequency of miniature EPSCs are higher in brain slices from mutants than controls
• spontaneous EPSC charge transfer is higher, whereas spontaneous IPSC change transfer is unaltered in mutants
• the frequency of miniature EPSCs is higher in brain slices from mutants than controls
• glutamatergic synapses show depression but no augmentation with repeated stimulation compared to wild-type mice which show neither facilitation nor depression and moderate augmentation

cellular
• mature granule cells show a persistently wide and heavily branched apical dendritic arbor typical of immature wild-type granule cells, indicating an inhibition of postnatal pruning of granule cell apical dendrites in mutants

Mouse Models of Human Disease
OMIM IDRef(s)
Epilepsy, Familial Temporal Lobe, 1; ETL1 600512 J:154129