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M_MECP2_57_KI_HE_ISOGUVACINE
Model Details
Change
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Phenotypic Profile
Category Entity Quantity Experimental Paradigm Age at Testing
Motor phenotype General locomotor activity: ambulatory activity1
Restored
Description: Isoguvacine treatment increased distance travelled to control levels.
Exp Paradigm: NA

Open field test 6 weeks
Motor phenotype General locomotor activity1
No adverse effect
Description: Isoguvacine treatment did not change total number of arm entries.
Exp Paradigm: NA

Elevated plus maze test 6 weeks
Neuroanatomy / Ultrastructure / Cytoarchitecture Neuronal number: interneurons1
Restored
Description: Isoguvacine increased parvalbumin positive interneurons in the primary somatosensory cortex but not in the primary visual cortex.
Exp Paradigm: NA

Immunohistochemistry 6 weeks
Neuroanatomy / Ultrastructure / Cytoarchitecture Neuronal number: interneurons1
Refractory
Description: Isoguvacine treatment did not change number of parvalbumin positive interneurons in the basolateral amygdala.
Exp Paradigm: NA

Immunohistochemistry 6 weeks
Neurophysiology Spontaneous post synaptic event amplitude: inhibitory currents1
No adverse effect
Description: Isoguvacine treatment did not change sipsc amplitude in the primary somatosensory cortex or the visual cortex.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Neurophysiology Synaptic transmission1
Restored
Description: Isoguvacine increased excitatory to inhibitory ratio in mutants.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Neurophysiology Spontaneous post synaptic event amplitude: excitatory currents1
No adverse effect
Description: Isoguvacine treatment did not change sepsc amplitude in the primary somatosensory cortex or the visual cortex.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Neurophysiology Spontaneous post synaptic event frequency: inhibitory currents1
Side effect
Description: Isoguvacine treatment increased sipsc frequency in the visual cortex but not the primary somatosensory cortex.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Neurophysiology Spontaneous post synaptic event frequency: excitatory currents1
No adverse effect
Description: Isoguvacine treatment did not change sepsc frequency in the primary somatosensory cortex or the visual cortex.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Neurophysiology Synaptic transmission1
Refractory
Description: Isoguvacine treatment did not change excitatory to inhibitory ratio in mutants.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Repetitive behavior Self grooming: perseveration1
No adverse effect
Description: Isoguvacine treatment did not change self-grooming.
Exp Paradigm: NA

Grooming behavior assessments 6 weeks
Repetitive behavior Repetitive digging1
Refractory
Description: Isoguvacine treatment did not change the number of marbles buried.
Exp Paradigm: NA

Marble-burying test 6 weeks
Sensory Sensorimotor gating1
Refractory
Description: Isoguvacine treatment did not change percent prepulse inhibition of the startle response to a 125 db noise, when the startle noise is preceded by a prepulse at 80db.
Exp Paradigm: NA

Prepulse inhibition 6 weeks
Sensory Startle response: acoustic stimulus1
No adverse effect
Description: Isoguvacine treatment did not change response to acoustic prepulse.
Exp Paradigm: NA

Acoustic startle reflex test 6 weeks
Sensory Sensorimotor gating: tactile cue1
Restored
Description: Isoguvacine decreased the increase of tactile prepulse inhibition of an acoustic startle response assay in mutants.
Exp Paradigm: NA

Prepulse inhibition 6 weeks
Sensory Tactile memory1
Refractory
Description: Isoguvacine treatment did not change preference for object with novel texture.
Exp Paradigm: NA

Textured novel object recognition test (t-nort) 6 weeks
Sensory Sensorimotor gating: tactile cue1
Restored
Description: Isoguvacine decreased response to air puff alone in mutants.
Exp Paradigm: NA

Response to air puff 6 weeks
Sensory Startle response: acoustic stimulus1
Refractory
Description: Isoguvacine treatment did not change startle response to 125db startle noise in mutants.
Exp Paradigm: NA

Acoustic startle reflex test 6 weeks
Social behavior Social memory1
Restored
Description: Isoguvacine increased preference for novel mouse over familiar mouse in mutants.
Exp Paradigm: NA

Three-chamber social approach test 6 weeks
Social behavior Social approach1
Restored
Description: Isoguvacine increased preference for novel mouse over novel object in mutants.
Exp Paradigm: NA

Three-chamber social approach test 6 weeks
Developmental profile Size/growth1
Restored
Description: Isoguvacine treatment increased body weight in mutants.
Exp Paradigm: NA

Body weight measurement NA
Developmental profile General characteristics1
Restored
Description: Mutants show clean shiny coat, clear eyes and normal stance
Exp Paradigm: A score of 0 indicated a clean shiny coat, clear eyes and normal stance. a score of 1 indicated the appearance of dull eyes, dull/ungroomed coat and somewhat hunched stance. a score of 2 denoted narrowed or crusted eyes, piloerection and a hunched posture. a score of 3 indicated severe eye narrowing, profound piloerection or loss of fur, and severely hunched posture.

Macroscopic analysis 3 weeks
Emotion Habituation to aversive stimuli1
Restored
Description: Isoguvacine treatment increased the decrease in startle amplitude.
Exp Paradigm: NA

Prepulse inhibition 6 weeks
Emotion Exploratory activity1
Restored
Description: Isoguvacine treatment decreased time spent investigating objects to control levels.
Exp Paradigm: NA

Novel object recognition test 6 weeks
Emotion Anxiety1
Restored
Description: Isoguvacine decreased time in the center in mutants.
Exp Paradigm: NA

Open field test 6 weeks
Emotion Anxiety1
Restored
Description: Isoguvacine decreased time in open arms in mutants.
Exp Paradigm: NA

Elevated plus maze test 6 weeks
Learning & memory Object recognition memory: long-term recall1
Refractory
Description: Isoguvacine treatment did not change preference for object with novel shape after 5 min or 1 hour retention.
Exp Paradigm: NA

Novel object recognition test 6 weeks
Not Reported: Circadian sleep/wake cycle,  Communications,  Immune response,  Maternal behavior,  Molecular profile,  Physiological parameters,  Seizure,  
 
References: 1:  Orefice LL , et al. 2019
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