• fewer than the expected number of male offspring are recovered from litters from crosses between cre-expressing males and Arx^tm1Gldn females (J:148311)

• a siginificant number of mutant males die early in the postnatal period (J:148311)

• some mice display seizures characterized by whole body flexion or extension movements resembling epileptic spasms (J:148311)

• some mice display arrest of acitivity/freezing seizures (J:148311)

• all adult mice develop spontaneous brief seizures (J:148311)

• all P14-17 mice demonstrate spontaneous seizures consisting of body arching with forelimb clonus and rearing (J:148311)

• no brain weight or gross morphological differences are detected in adult or P14-17 animals compared to controls (J:148311)

• some mice display seizures characterized by whole body flexion or extension movements resembling epileptic spasms (J:148311)

• some mice display arrest of acitivity/freezing seizures (J:148311)

• all adult mice develop spontaneous brief seizures (J:148311)

• all P14-17 mice demonstrate spontaneous seizures consisting of body arching with forelimb clonus and rearing (J:148311)

• a prominent reduction in calbindin-labeled neurons in the neocortex compared to controls in the hippocampus, pattern of staining of interneurons is altered from cell body staining to mainly staining interneuron processes (J:148311)

• smaller reductions are observed in numbers and distribution of calretinin-labeled neurons compared to controls (J:148311)

• in P90-120 day-old mice, EEG is disrupted compared to controls; mice display pattern of moderate to higher amplitude and faster frequency activity (J:148311)

• abnormal activity is more apparent in the hippocampal rather than cortical electrodes (J:148311)

• adult animals show a lack of normal 4-7 Hz rhythmic theta activity while awake; hippocampal theta activity is rarely recorded, but when present has faster activity superimposed on the normal theta (J:148311)

• while sleeping, a lack of normal delta power and rhythmic delta activity seen in controls (J:148311)

• mice have a decrease in delta activity and an increase in faster frequency activity (J:148311)

• P14-17 animals display EEGs with slower background with lower voltage than mature controls; one animal showed infrequent large amplitude spikes (J:148311)

• some adult mice develop convulsive Racine stage 5 seizures (J:148311)

• some adult mice display epileptic spasm seizures (J:148311)

• some adult mice display arrest of acitivity/freezing seizures (J:148311)

• about half of adult female mice develop spontaneous brief seizures of various types (J:148311)

• P14-17 mice develop seizures at a similar rate to adult females; no epileptic spasm seizures are recorded in mice at this ages (J:148311)

• no brain weight or gross morphological differences are detected in adult or P14-17 animals compared to controls (J:148311)

• some adult mice develop convulsive Racine stage 5 seizures (J:148311)

• some adult mice display epileptic spasm seizures (J:148311)

• some adult mice display arrest of acitivity/freezing seizures (J:148311)

• about half of adult female mice develop spontaneous brief seizures of various types (J:148311)

• P14-17 mice develop seizures at a similar rate to adult females; no epileptic spasm seizures are recorded in mice at this ages (J:148311)

• a prominent reduction in calbindin-labeled neurons in the neocortex compared to controls in the hippocampus, pattern of staining of interneurons is altered from cell body staining to mainly staining interneuron processes (J:148311)

• a significant reduction is observed in numbers and distribution of calretinin-labeled neurons compared to controls (J:148311)

• EEGs of female mice exhibit abnormal background activity; EEG is periodically interrupted by longer runs of higher amplitude, faster rhythms, and demonstrates excessive sharp activity (J:148311)

• females show no decrease in Delta band activity, but do exhibit an increase in faster frequency activity like male mutants (J:148311)

• the thalamocortical axons aberrantly run through the pallidum and amygdala

• while corticofugal fibers cross the pallial-subpallial boundary and enter the lateral part of the basal forebrain, they fail to progress and spiral in a disordered manner

• both corticothalamic and thalamocortial axons are abnormal

• in the Morris water maze, mutants have a longer latency to locate the platform on the first 2 days of training than controls, however performance on the probe test and reversal probe test were normal, indicating normal hippocampal-mediated spatial learning (J:170554)

• open field activity and anxiety are not affected (J:170554)

• on reversal discriminations, mutants require more trials than controls, indicating impaired reversal learning

• mutants show increased latency performance on the cued-platform test, indicating a delay in striatal-dependent cued learning

• marker analysis indicates an increase in numbers of PV+ and SST+ striatal GABAergic interneurons

• distribution of striatal interneurons is altered, with fewer cells of the population located in the medial (associative) regions and more cells in the lateral (sensorimotor) areas

• reduction in the numbers of PV+ GABAergic interneurons in the primary somatosensory cortex (28% loss) and the orbitofrontal cortex (31% loss)

• marker analysis indicates an increase in numbers of PV+ and SST+ striatal GABAergic interneurons

• however, striatum appears normal

• 31% reduction in PV+ cells in the orbitofrontal cortex

• 28% loss of PV+ cells in primary somatosensory cortex

• most mice do not exhibit exencephaly (J:107576)

• reduced in size

• stacked arrangement of glomeruli

• lacking

• noticably smaller

• viable with no gross phenotype

• cortical interneuron profiles are normal