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construct
M_MECP2_52_CKO_HE
Model Details
Change
No Change
     
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 6-8 weeks
Neuroanatomy / Ultrastructure / Cytoarchitecture Neuronal number: interneurons1
Increased
Description: Mutants show increase in number of parvalbumin positive interneurons in primary somatosensory cortex, but not primary visual cortex. mutants show decrease in number of parvalbumin positive interneurons in the basolateral amygdala.
Exp Paradigm: Primary somatosensory cortex, primary visual cortex

Immunohistochemistry Adult
Neuroanatomy / Ultrastructure / Cytoarchitecture Morphology of the spinal cord: sensory and motor tracts1
Decreased
Description: Mutants show decrease in hcn1 expression at spinal cord dorsal horn lamina iii/iv in vglut1+ presynaptic terminals, in large but not small diameter drg cell bodies.
Exp Paradigm: Spinal cord

Immunohistochemistry Adult
Neuroanatomy / Ultrastructure / Cytoarchitecture Neuroreceptor levels: gaba-r: gabaa1
Decreased
Description: Mutants show decrease in the levels of gabrb3 expression in low-threshold mechanoreceptor neuron (ltmr) central terminals at spinal cord dorsal horn lamina iii/iv in vglut1+ presynaptic terminals.
Exp Paradigm: Spinal cord

Immunohistochemistry Adult
Neurophysiology Spontaneous post synaptic event frequency: inhibitory currents1
Increased
Description: Mutants show increase in spontaneous inhibitory postsynaptic current (sipsc) frequency in layer 2/3 pyramidal neurons from acute primary somatosensory cortex slices resulting in a decrease in e/i ratio. mutants show no change in spontaneous inhibitory postsynaptic current (sipsc) frequency in the visual cortex.
Exp Paradigm: Primary somatosensory cortex, primary visual cortex

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

Marble-burying test 6-8 weeks
Sensory Sensorimotor gating: tactile cue1
Increased
Description: Mutants exhibited hairy skin hypersensitivity, measured by an increase of tactile prepulse inhibition of an acoustic startle response assay.
Exp Paradigm: NA

Prepulse inhibition 6-8 weeks
Sensory Tactile memory1
Decreased
Description: Mutants show decrease in preference for the object with the novel texture.
Exp Paradigm: NA

Textured novel object recognition test (t-nort) 6-8 weeks
Sensory Sensorimotor gating: tactile cue1
Increased
Description: Mutants show increased responsivity to an air puff stimulus delivered to back hairy skin.
Exp Paradigm: NA

Response to air puff 6-8 weeks
Social behavior Social approach1
Decreased
Description: Mutants spent less time investigating a novel mouse than a novel object.
Exp Paradigm: NA

Three-chamber social approach test 6-8 weeks
Social behavior Social memory1
Decreased
Description: Mutants spent less time investigating a novel mouse than a familiar mouse.
Exp Paradigm: NA

Three-chamber social approach test 6-8 weeks
Emotion Anxiety1
Increased
Description: Mutants spent less time in the open arms.
Exp Paradigm: NA

Elevated plus maze test 6-8 weeks
Emotion Anxiety1
Increased
Description: Mutants spent less time in the center of the open field.
Exp Paradigm: NA

Open field test 6-8 weeks
Emotion Habituation to aversive stimuli1
Decreased
Description: Mutants show lower percent decrease in startle response 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 for mutant mice and their control littermates, indicating lack of habituation to an acoustic startle noise.
Exp Paradigm: NA

Prepulse inhibition 6-8 weeks
Emotion Exploratory activity1 No change Novel object recognition test 6-8 weeks
Learning & memory Object recognition memory: long-term recall1 No change Novel object recognition test 6-8 weeks
Motor phenotype General locomotor activity1 No change Elevated plus maze test 6-8 weeks
Neurophysiology Spontaneous post synaptic event amplitude: excitatory currents1 No change Whole-cell patch clamp Adult
Neurophysiology Spontaneous post synaptic event amplitude: inhibitory currents1 No change Whole-cell patch clamp Adult
Neurophysiology Spontaneous post synaptic event frequency: excitatory currents1 No change Whole-cell patch clamp Adult
Repetitive behavior Self grooming: perseveration1 No change Open field test 6-8 weeks
Sensory Sensorimotor gating1 No change Prepulse inhibition 6-8 weeks
Sensory Startle response: acoustic stimulus1 No change Acoustic startle reflex test 6-8 weeks
Not Reported: Circadian sleep/wake cycle,  Communications,  Developmental profile,  Immune response,  Maternal behavior,  Molecular profile,  Physiological parameters,  Seizure,  
 
References: 1:  Orefice LL , et al. 2019
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