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M_MECP2_57_KI_HE
Model Details
Change
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Phenotypic Profile
Category Entity Quantity Experimental Paradigm Age at Testing
Motor phenotype General locomotor activity: ambulatory activity1
Decreased
Description: Mutants show decrease in distance travelled.
Exp Paradigm: NA

Open field test 6 weeks
Neuroanatomy / Ultrastructure / Cytoarchitecture Neuronal number: interneurons1
Increased
Description: Mutants show increased parvalbumin positive neurons in the primary somatosensory cortex and in the visual cortex but not in the basolateral amygdala.
Exp Paradigm: NA

Immunohistochemistry 6 weeks
Neuroanatomy / Ultrastructure / Cytoarchitecture Neuronal number: interneurons1
Decreased
Description: Mutants show decrease in the number of interneurons in the basolateral amygdala.
Exp Paradigm: NA

Immunohistochemistry 6 weeks
Neuroanatomy / Ultrastructure / Cytoarchitecture Neuroreceptor levels: gaba-r: gabaa1
Decreased
Description: Mutants show decreased expression of gabrb3 expression in the spinal cord dorsal horn lamina iii/iv.
Exp Paradigm: Spinal cord

Immunohistochemistry 6 weeks
Neurophysiology Synaptic transmission1
Decreased
Description: Mutants show decrease in excitatory to inhibitory ratio in primary somatosensory cortex slices.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Neurophysiology Spontaneous post synaptic event frequency: inhibitory currents1
Abnormal
Description: Mutants show increase in sipsc frequency in the primary visual cortex but not in the somatosensory cortex.
Exp Paradigm: NA

Whole-cell patch clamp 6 weeks
Neurophysiology Synaptic transmission1
Decreased
Description: Mutants show decrease in excitatory to inhibitory ratio in primary somatosensory cortex slices.
Exp Paradigm: Primary somatosensory cortex

Whole-cell patch clamp 6 weeks
Repetitive behavior Repetitive digging1
Decreased
Description: Mutants show decrease in the number of marbles buried.
Exp Paradigm: NA

Marble-burying test 6 weeks
Sensory Sensorimotor gating: tactile cue1
Increased
Description: Mutants show increase in response to air puff.
Exp Paradigm: NA

Response to air puff 6 weeks
Sensory Tactile memory1
Decreased
Description: Mutants show decreased preference for object with novel texture.
Exp Paradigm: NA

Textured novel object recognition test (t-nort) 6 weeks
Sensory Sensorimotor gating1
Increased
Description: Mutants show increased 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
Decreased
Description: Mutants show decrease in amplitude of startle response to a 125 db startling noise but not to a 80db non-startling noise pulse.
Exp Paradigm: NA

Acoustic startle reflex test 6 weeks
Sensory Sensorimotor gating: tactile cue1
Increased
Description: Mutants show increased percent inhibition of the startle response to a 125 db noise, when the startle noise is preceded by a light air puff.
Exp Paradigm: NA

Prepulse inhibition 6 weeks
Social behavior Social memory1
Decreased
Description: Mutants show decrease in preference for novel mouse over familiar mouse.
Exp Paradigm: NA

Three-chamber social approach test 6 weeks
Social behavior Social approach1
Decreased
Description: Mutants show decrease in the preference for novel mouse over novel object.
Exp Paradigm: NA

Three-chamber social approach test 6 weeks
Social behavior Social approach1
Decreased
Description: Mutants show decrease in preference for novel mouse over novel object.
Exp Paradigm: NA

Three-chamber social approach test 6 weeks
Developmental profile Size/growth1
Decreased
Description: Mutants show reduced body weight.
Exp Paradigm: NA

Body weight measurement NA
Developmental profile General characteristics1
Decreased
Description: Mutants show dull eyes, dull/ungroomed coat and somewhat hunched 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
Decreased
Description: Mutants show decreased startle amplitude to a 125 db noise during a 30-minute tactile ppi session, when comparing the first five startle responses to the last five responses to a 125 db noise.
Exp Paradigm: NA

Prepulse inhibition 6 weeks
Emotion Exploratory activity1
Increased
Description: Mutants show increase in time spent investigating objects.
Exp Paradigm: NA

Novel object recognition test 6 weeks
Emotion Anxiety1
Increased
Description: Mutants show decrease in time spent in the open arms.
Exp Paradigm: NA

Elevated plus maze test 6 weeks
Emotion Anxiety1
Increased
Description: Mutants show decrease in time spent in the center.
Exp Paradigm: NA

Open field test 6 weeks
Learning & memory Object recognition memory: long-term recall1
Decreased
Description: Mutants show decrease in preference for object with novel shape after 1 hour retention but not after 5 min retention.
Exp Paradigm: NA

Novel object recognition test 6 weeks
Emotion Exploratory activity1 No change Novel object recognition test 6 weeks
Motor phenotype General locomotor activity1 No change Elevated plus maze test 6 weeks
Neurophysiology Spontaneous post synaptic event amplitude: excitatory currents1 No change Whole-cell patch clamp 6 weeks
Neurophysiology Spontaneous post synaptic event amplitude: inhibitory currents1 No change Whole-cell patch clamp 6 weeks
Neurophysiology Spontaneous post synaptic event frequency: excitatory currents1 No change Whole-cell patch clamp 6 weeks
Repetitive behavior Self grooming: perseveration1 No change Grooming behavior assessments 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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