cellular phenotype

• abnormal neuron differentiation
  • development of GABAergic neurons is accelerated in the primary visual cortex of transgenic mice between P12 and P35, relative to wild-type mice
  (MGI Ref ID J:57645)

vision/eye phenotype

• abnormal eye physiology
  • visual acuity is accelerated in transgenic mice relative to wild-type; visual acuity reaches adult levels by P24-25, a week sooner than in wild-type
  (MGI Ref ID J:57645)

mammalian phenotype

• nervous system phenotype
  • brain development at 4 weeks is grossly normal; cortex is of normal thickness, and lamination is intact with normal neuronal cell density in the visual cortex
  (MGI Ref ID J:57645)

nervous system phenotype

• abnormal brain interneuron morphology
  • these interneurons have larger soma sizes and more extensive neurite arborizations than in wild-type at P17
  (MGI Ref ID J:57645)
  • at P17, number of Parvalbumin-positive interneurons in the visual cortex is ~double the number found in wild-type brains
  (MGI Ref ID J:57645)

• abnormal inhibitory postsynaptic currents
  • stimulus intensity required to recruit a saturating IPSC is similar in wild-type and transgenic brain slices
  (MGI Ref ID J:57645)
  • EPSC amplitudes and whole-cell capacitances are similar between transgenic and wild-type mice
  (MGI Ref ID J:57645)
  • in layer III pyramidal neurons in hippoccampal slices, amplitude of the maximal IPSC (462 pA) is consistently large than in wild-type (279 pA)
  (MGI Ref ID J:57645)

• abnormal neuron differentiation
  • development of GABAergic neurons is accelerated in the primary visual cortex of transgenic mice between P12 and P35, relative to wild-type mice
  (MGI Ref ID J:57645)

• reduced long term potentiation
  • theta burst stimulation (TBS) at the white matter-layer IV border (WM->III) induces little LTP in transgenic mice (101%), compared to the robust LTP induced in wild-type mice (122%)
  (MGI Ref ID J:57645)
  • when inhibition is induced in cortical slices from transgenic mice with picrotoxin application, WM->III LTP can be supported by mice at P23-P25
  (MGI Ref ID J:57645)
  • WM->III LTP undergoes a sharp decline between 3 and 4 weeks postnatal, which is 1 week earlier than observed in wild-type littermates (between 4 and 5 weeks of age)
  (MGI Ref ID J:57645)

• abnormal visual cortex morphology
  • ocular dominance (OD) plasticity ends prematurely in transgenic mice relative to wild-type; the OD shift induced by 4-day monocular deprivation (MD) is blocked when done at P28, near the peak of OD shift in wild-type
  (MGI Ref ID J:57645)
  • shifts in the contralateral/ipsilateral visual-evoked potential (C/I VEP) ratios are larger in transgenics when MD is done with a difference of ~1 week compared to wild-type
  (MGI Ref ID J:57645)

• abnormal neocortex morphology
  • at 5 weeks, BDNF mRNA levels in neocortex of transgenic mice are ~3-fold higher than in wild-type littermates
  (MGI Ref ID J:57645)

behavior/neurological phenotype

• abnormal behavior
  • Normal - however, mice show normal behavior in the social approach test and in the forced swim test
  (MGI Ref ID J:216846)
  • mice bury less marbles than wild-type mice
  (MGI Ref ID J:216846)

• seizures
  • mice exhibit spontaneous seizures after 5 months of age, with 3 of 12 mice showing seizures at 9 months of age
  (MGI Ref ID J:216846)
  • seizure behavior includes head bobbing, forelimb and hindlimb clonus that progresses into running and jumping seizures followed by falling and the continuation of head bobbing and forelimb clonus
  (MGI Ref ID J:216846)

• psychomotor seizures
  • seizures are similar to stage 5 limbic seizures
  (MGI Ref ID J:216846)

• decreased anxiety-related response
  • mice spend more time in the open arms of the elevated plus-maze and make more entries to the open arms than wild-type mice
  (MGI Ref ID J:216846)

growth/size/body region phenotype

• decreased body weight
  • mice show decreased body weight that becomes more pronounced with age
  (MGI Ref ID J:216846)

nervous system phenotype

• psychomotor seizures
  • seizures are similar to stage 5 limbic seizures
  (MGI Ref ID J:216846)

• seizures
  • seizure behavior includes head bobbing, forelimb and hindlimb clonus that progresses into running and jumping seizures followed by falling and the continuation of head bobbing and forelimb clonus
  (MGI Ref ID J:216846)
  • mice exhibit spontaneous seizures after 5 months of age, with 3 of 12 mice showing seizures at 9 months of age
  (MGI Ref ID J:216846)