| Phenotypic Profile |
| Category |
Entity |
Quantity |
Experimental Paradigm |
Age at Testing |
| Motor phenotype |
General locomotor activity1 |
Decreased
Description: Decreased voluntary locomotor activity
Exp Paradigm: Voluntary wheel-running activity
|
Running wheel test |
8 weeks |
| |
|
Description: Decreased voluntary locomotor activity
Exp Paradigm: Voluntary wheel-running activity |
|
|
| Motor phenotype |
Motor coordination and balance2 |
Decreased
Description: Decreased motor coordination
Exp Paradigm: Rotarod test
|
Accelerating rotarod test |
6 weeks |
| |
|
Description: Decreased motor coordination
Exp Paradigm: Rotarod test |
|
|
| Motor phenotype |
General locomotor activity2 |
Decreased
Description: Decreased locomotor activity with steady hypoactivity across testing period
Exp Paradigm: General motor activity levels during dark cycle
|
Light-dark exploration test |
3, 4, 6 weeks |
| |
|
Description: Decreased locomotor activity with steady hypoactivity across testing period
Exp Paradigm: General motor activity levels during dark cycle |
|
|
| Motor phenotype |
Grip strength2 |
Decreased
Description: Decreased forepaw grip strength
Exp Paradigm: Grip strength assay
|
Grip strength test |
3, 4, 6 weeks |
| |
|
Description: Decreased forepaw grip strength
Exp Paradigm: Grip strength assay |
|
|
| Motor phenotype |
Gait3 |
Decreased
Description: Mecp2 homozygous null mice show decreased gait score compared to previously published controls.
Exp Paradigm: Walking gait was assessed using an observational scoring system while mice walked on a flat table top. a score of 0 indicated normal gait, 1 indicated hind legs spread wider than normal when ambulating, 2 indicated severe abnormality either with tremor when feet were lifted or with backwards walking by lifting both rear feet simultaneously.
|
Gait |
4 months |
| |
|
Description: Mecp2 homozygous null mice show decreased gait score compared to previously published controls.
Exp Paradigm: Walking gait was assessed using an observational scoring system while mice walked on a flat table top. a score of 0 indicated normal gait, 1 indicated hind legs spread wider than normal when ambulating, 2 indicated severe abnormality either with tremor when feet were lifted or with backwards walking by lifting both rear feet simultaneously. |
|
|
| Motor phenotype |
General locomotor activity: ambulatory activity3 |
Decreased
Description: Mecp2 homozygous null mice show reduced number of beam breaks compared to previously published controls.
Exp Paradigm: Locomotor activity was determined for 8 hours during the active nocturnal phase of mice.
|
Open field test |
4 months |
| |
|
Description: Mecp2 homozygous null mice show reduced number of beam breaks compared to previously published controls.
Exp Paradigm: Locomotor activity was determined for 8 hours during the active nocturnal phase of mice. |
|
|
| Neuroanatomy / Ultrastructure / Cytoarchitecture |
Dendritic architecture: spine density2 |
Abnormal
Description: Abnormal dendritic cell morphology indicated by smaller cell body size and shorter dendritic lengths
Exp Paradigm: Sholl analysis of cell morphology in the dentate gyrus of the hippocampus
|
Sholl analysis |
Unreported |
| |
|
Description: Abnormal dendritic cell morphology indicated by smaller cell body size and shorter dendritic lengths
Exp Paradigm: Sholl analysis of cell morphology in the dentate gyrus of the hippocampus |
|
|
| Neuroanatomy / Ultrastructure / Cytoarchitecture |
Brain cytoarchitecture1 |
Decreased
Description: Decreased hippocampal neuronal nuclei size
Exp Paradigm: Ca1 neuronal nuclei size measurement
|
Nuclear size measurement |
8-10 weeks |
| |
|
Description: Decreased hippocampal neuronal nuclei size
Exp Paradigm: Ca1 neuronal nuclei size measurement |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins2 |
Increased
Description: Increased expression of ngf is seen in the striatum
Exp Paradigm: Bdnf/ngf elisa's
|
Immunohistochemistry |
6 weeks |
| |
|
Description: Increased expression of ngf is seen in the striatum
Exp Paradigm: Bdnf/ngf elisa's |
|
|
| Neurophysiology |
Presynaptic function: paired-pulse facilitation3 |
Decreased
Description: Mecp2 homozygous null mutants show decreased ppr compared to controls.
Exp Paradigm: An input stimulus intensity that induces 4060% of the maximum response was used for measuring paired-pulse ratios (ppr). ppr was measured by giving 2 pulses at decreasing interstimulus intervals (500, 400, 200, 100, 50, 30, 20 ms) and analyzed by dividing the fepsp slope of pulse 2 by pulse 1.
|
Field potential recordings |
4 months |
| |
|
Description: Mecp2 homozygous null mutants show decreased ppr compared to controls.
Exp Paradigm: An input stimulus intensity that induces 4060% of the maximum response was used for measuring paired-pulse ratios (ppr). ppr was measured by giving 2 pulses at decreasing interstimulus intervals (500, 400, 200, 100, 50, 30, 20 ms) and analyzed by dividing the fepsp slope of pulse 2 by pulse 1. |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins2 |
Decreased
Description: Abnormal bdnf/ngf expression with decreased levels of bdnf in cerebellum and increased ngf in striatum
Exp Paradigm: Bdnf/ngf elisa's
|
Immunohistochemistry |
NA |
| |
|
Description: Abnormal bdnf/ngf expression with decreased levels of bdnf in cerebellum and increased ngf in striatum
Exp Paradigm: Bdnf/ngf elisa's |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins3 |
Decreased
Description: Mecp2 homozygous null mice displayed reduced amount of total bdnf protein in the brainstem and striatum compared to controls. mecp2 homozygous null mice displayed reduced amount of mature bdnf in the brainstem, striatum and hippocampus compared to controls. mecp2 homozygous null mice displayed reduced amounts of pro-bdnf in the brainstem and striatum compared to controls.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa
|
Elisa |
4 months |
| |
|
Description: Mecp2 homozygous null mice displayed reduced amount of total bdnf protein in the brainstem and striatum compared to controls. mecp2 homozygous null mice displayed reduced amount of mature bdnf in the brainstem, striatum and hippocampus compared to controls. mecp2 homozygous null mice displayed reduced amounts of pro-bdnf in the brainstem and striatum compared to controls.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins3 |
Decreased
Description: Mecp2 homozygous null mice displayed reduced amount of total bdnf protein in the brainstem and striatum compared to controls. mecp2 homozygous null mice displayed reduced amount of mature bdnf in the brainstem, striatum and hippocampus compared to controls. mecp2 homozygous null mice displayed reduced amounts of pro-bdnf in the brainstem and striatum compared to controls.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.- western blot
|
Western blot |
4 months |
| |
|
Description: Mecp2 homozygous null mice displayed reduced amount of total bdnf protein in the brainstem and striatum compared to controls. mecp2 homozygous null mice displayed reduced amount of mature bdnf in the brainstem, striatum and hippocampus compared to controls. mecp2 homozygous null mice displayed reduced amounts of pro-bdnf in the brainstem and striatum compared to controls.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.- western blot |
|
|
| Neurophysiology |
Synaptic plasticity: cerebellar ltp |
Decreased
Description: Mecp2 homozygous null mice show decreased high-frequency induced ltp in hippocampal slices compared to controls.
Exp Paradigm: An input stimulus intensity that induces 4060% of the maximum response was used for measuring long-term potentiation (ltp). after 20 minutes of stable baseline, ltp was induced by high frequency stimulation using an input stimulus intensity that produces the maximum response (hfs, 2 100 hz trains with 100 pulses with an inter- burst interval of 20 seconds).
|
Field potential recordings |
4 months |
| |
|
Description: Mecp2 homozygous null mice show decreased high-frequency induced ltp in hippocampal slices compared to controls.
Exp Paradigm: An input stimulus intensity that induces 4060% of the maximum response was used for measuring long-term potentiation (ltp). after 20 minutes of stable baseline, ltp was induced by high frequency stimulation using an input stimulus intensity that produces the maximum response (hfs, 2 100 hz trains with 100 pulses with an inter- burst interval of 20 seconds). |
|
|
| Physiological parameters |
Respiratory function1 |
Increased
Description: Increased ventilatory capacity as measured by tidal volume and minute pulmonary ventilation
Exp Paradigm: Whole-body plesmythographic measurements
|
Whole body plethysmography |
6 weeks |
| |
|
Description: Increased ventilatory capacity as measured by tidal volume and minute pulmonary ventilation
Exp Paradigm: Whole-body plesmythographic measurements |
|
|
| Physiological parameters |
Respiratory function1 |
Abnormal
Description: Abnormal breathing pattern as indicated by increased total duration of each breath and greater confidence interval and coefficient of variation
Exp Paradigm: Whole-body plesmythographic measurements
|
Whole body plethysmography |
6 weeks |
| |
|
Description: Abnormal breathing pattern as indicated by increased total duration of each breath and greater confidence interval and coefficient of variation
Exp Paradigm: Whole-body plesmythographic measurements |
|
|
| Physiological parameters |
Metabolic function2 |
Decreased
Description: Decreased metabolic function indicated by decreased co2 production and lower o2 consumption
Exp Paradigm: Gas exchange measurements
|
Measurement of gas exchange |
6 weeks |
| |
|
Description: Decreased metabolic function indicated by decreased co2 production and lower o2 consumption
Exp Paradigm: Gas exchange measurements |
|
|
| Physiological parameters |
Respiratory function3 |
Decreased
Description: Mecp2 homozygous null mice show increased incidence of apnea compared to previously published controls.
Exp Paradigm: Apneas were defined as a pause in the breathing cycle lasting longer than 1.5 seconds.
|
Whole body plethysmography |
4 months |
| |
|
Description: Mecp2 homozygous null mice show increased incidence of apnea compared to previously published controls.
Exp Paradigm: Apneas were defined as a pause in the breathing cycle lasting longer than 1.5 seconds. |
|
|
| Developmental profile |
Mortality/lethality1 |
Increased
Description: Increased lethality
Exp Paradigm: General observations
|
General observations |
7.5 days |
| |
|
Description: Increased lethality
Exp Paradigm: General observations |
|
|
| Developmental profile |
Size/growth2 |
Decreased
Description: Decreased rate of weight gain starting just after weaning
Exp Paradigm: General observations
|
General observations |
3 weeks |
| |
|
Description: Decreased rate of weight gain starting just after weaning
Exp Paradigm: General observations |
|
|
| Developmental profile |
Mortality/lethality1 |
Increased
Description: Decreased lifespan
Exp Paradigm: General observations
|
General observations |
Unreported |
| |
|
Description: Decreased lifespan
Exp Paradigm: General observations |
|
|
| Developmental profile |
Size/growth1 |
Decreased
Description: Decreased body weight
Exp Paradigm: General observations
|
General observations |
4-8 weeks |
| |
|
Description: Decreased body weight
Exp Paradigm: General observations |
|
|
| Developmental profile |
Mortality/lethality3 |
Increased
Description: Mecp2 homozygous null mice show increased lethality compared to controls.
Exp Paradigm: NA
|
General observations |
4 months |
| |
|
Description: Mecp2 homozygous null mice show increased lethality compared to controls.
Exp Paradigm: NA |
|
|
| Learning & memory |
Cued or contextual fear conditioning2 |
Abnormal
Description: Abnormal cued/contextual conditioning demonstrated by deficit in cued but not contextual fear conditioning
Exp Paradigm: Associative fear conditioning task
|
Fear conditioning test |
6 weeks |
| |
|
Description: Abnormal cued/contextual conditioning demonstrated by deficit in cued but not contextual fear conditioning
Exp Paradigm: Associative fear conditioning task |
|
|
| Learning & memory |
Object recognition memory2 |
Decreased
Description: Decreased long term memory demonstrated by no memory for object 1
Exp Paradigm: Object recognition task
|
Novel object recognition test |
4 weeks |
| |
|
Description: Decreased long term memory demonstrated by no memory for object 1
Exp Paradigm: Object recognition task |
|
|
| Developmental profile |
General characteristics3 |
No change |
General observations |
4 months |
| Developmental profile |
Size/growth3 |
No change |
Body weight measurement |
1-4 months |
| Learning & memory |
Object recognition memory2 |
No change |
Novel object recognition test |
4 weeks |
| Motor phenotype |
Clasping reflex3 |
No change |
General observations |
4 months |
| Motor phenotype |
Grip strength3 |
No change |
Grip strength test |
4 months |
| Motor phenotype |
Tremor3 |
No change |
Tremor activity measurements |
4 months |
| Neurophysiology |
Epsp-spike relationship |
No change |
Field potential recordings |
4 months |
| Neurophysiology |
Neurotransmitter release: neurotrophins3 |
No change |
Western blot |
4 months |
| Neurophysiology |
Neurotransmitter release: neurotrophins3 |
No change |
Elisa |
4 months |
| Physiological parameters |
Metabolic function2 |
No change |
Measurement of gas exchange |
3, 4 weeks |
|
| Not Reported: |
Circadian sleep/wake cycle, Communications, Emotion, Immune response, Learning & memory, Maternal behavior, Molecular profile, Neuroanatomy / ultrastructure / cytoarchitecture, Neurophysiology, Physiological parameters, Repetitive behavior, Seizure, Sensory, Social behavior, |
