| Phenotypic Profile |
| Category |
Entity |
Quantity |
Experimental Paradigm |
Age at Testing |
| Motor phenotype |
General locomotor activity: ambulatory activity1 |
Ameliorated
Description: Dcs treatment increased the number of beam breaks due to increased locomotion in mecp2 null mutant mice.
Exp Paradigm: Locomotor activity was determined for 8 hours during the active nocturnal phase of mice.
|
Open field test |
4 months |
| |
|
Description: Dcs treatment increased the number of beam breaks due to increased locomotion in mecp2 null mutant mice.
Exp Paradigm: Locomotor activity was determined for 8 hours during the active nocturnal phase of mice. |
|
|
| Motor phenotype |
Gait1 |
No adverse effect
Description: Dcs treatment showed no change in gait in mecp2 mutants.
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: Dcs treatment showed no change in gait in mecp2 mutants.
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 |
Tremor1 |
No adverse effect
Description: Dcs treatment showed no change in tremor in mecp2 mutants.
Exp Paradigm: Tremor was measured on a qualitative observational scoring system, a score of 0 indicating no tremor, 1 indicating mild intermittent tremor, and 2 indicating constant tremor or intermittent severe tremor.
|
Tremor activity measurements |
4 months |
| |
|
Description: Dcs treatment showed no change in tremor in mecp2 mutants.
Exp Paradigm: Tremor was measured on a qualitative observational scoring system, a score of 0 indicating no tremor, 1 indicating mild intermittent tremor, and 2 indicating constant tremor or intermittent severe tremor. |
|
|
| Motor phenotype |
Clasping reflex1 |
No adverse effect
Description: Dcs treatment showed no change in clasping in mecp2 mutants.
Exp Paradigm: Hind limb clasping was defined as when the hind limbs were spread in the area less than their body width while the animal was suspended by its tail. no clasping received a score of 0, and clasping received a score of 1.
|
General observations |
4 months |
| |
|
Description: Dcs treatment showed no change in clasping in mecp2 mutants.
Exp Paradigm: Hind limb clasping was defined as when the hind limbs were spread in the area less than their body width while the animal was suspended by its tail. no clasping received a score of 0, and clasping received a score of 1. |
|
|
| Motor phenotype |
Grip strength1 |
No adverse effect
Description: Dcs treatment showed no change in grip strength in mecp2 mutants.
Exp Paradigm: Mice were suspended by their forelimbs above standard cage bedding, and time to fall was recorded.
|
Grip strength test |
4 months |
| |
|
Description: Dcs treatment showed no change in grip strength in mecp2 mutants.
Exp Paradigm: Mice were suspended by their forelimbs above standard cage bedding, and time to fall was recorded. |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins1 |
Ameliorated
Description: Dcs treatment increased total bdnf levels in the brainstem and the striatum in mecp2 mutants. dcs treatment increased pro bdnf levels in the brainstem and striatum in mecp2 mutants.
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: Dcs treatment increased total bdnf levels in the brainstem and the striatum in mecp2 mutants. dcs treatment increased pro bdnf levels in the brainstem and striatum in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.- western blot |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins1 |
No adverse effect
Description: Dcs treatment did not change total bdnf or probdnf levels in the hippocampus in mecp2 mutants.
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: Dcs treatment did not change total bdnf or probdnf levels in the hippocampus in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.- western blot |
|
|
| Neurophysiology |
Synaptic plasticity: cerebellar ltp1 |
Refractory
Description: Dcs treatment did not improve the reduced hippocampal ltp in mecp2 null mice.
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: Dcs treatment did not improve the reduced hippocampal ltp in mecp2 null mice.
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). |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins1 |
Refractory
Description: Dcs treatment did not increase the levels of mature bdnf in the brainstem, striatum or hippocampus in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa
|
Elisa |
4 months |
| |
|
Description: Dcs treatment did not increase the levels of mature bdnf in the brainstem, striatum or hippocampus in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa |
|
|
| Neurophysiology |
Presynaptic function: paired-pulse facilitation1 |
Ameliorated
Description: Dcs treatment reversed paired pulse ratios at 20, 30, and 50 millisec interstimulus intervals in mecp2 null mutants suggesting dcs ameliorates ppr deficits at these intervals.
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 millisecond) and analyzed by dividing the fepsp slope of pulse 2 by pulse 1.
|
Field potential recordings |
4 months |
| |
|
Description: Dcs treatment reversed paired pulse ratios at 20, 30, and 50 millisec interstimulus intervals in mecp2 null mutants suggesting dcs ameliorates ppr deficits at these intervals.
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 millisecond) and analyzed by dividing the fepsp slope of pulse 2 by pulse 1. |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins1 |
Refractory
Description: Dcs treatment did not increase the levels of mature bdnf in the brainstem, striatum or hippocampus in mecp2 mutants.
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: Dcs treatment did not increase the levels of mature bdnf in the brainstem, striatum or hippocampus in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.- western blot |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins1 |
Ameliorated
Description: Dcs treatment increased total bdnf levels in the brainstem and the striatum in mecp2 mutants. dcs treatment increased pro bdnf levels in the brainstem and striatum in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa
|
Elisa |
4 months |
| |
|
Description: Dcs treatment increased total bdnf levels in the brainstem and the striatum in mecp2 mutants. dcs treatment increased pro bdnf levels in the brainstem and striatum in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa |
|
|
| Neurophysiology |
Neurotransmitter release: neurotrophins1 |
No adverse effect
Description: Dcs treatment did not change total bdnf or probdnf levels in the hippocampus in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa
|
Elisa |
4 months |
| |
|
Description: Dcs treatment did not change total bdnf or probdnf levels in the hippocampus in mecp2 mutants.
Exp Paradigm: Total, mature and pro-bdnf protein levels was measured with recombinant mature bdnf as a standard.-elisa |
|
|
| Physiological parameters |
Respiratory function1 |
Ameliorated
Description: Dcs treatment showed reduced incidence of apnea compared to mecp2 null mice.
Exp Paradigm: Apneas were defined as a pause in the breathing cycle lasting longer than 1.5 seconds.
|
Whole body plethysmography |
4 months |
| |
|
Description: Dcs treatment showed reduced incidence of apnea compared to mecp2 null mice.
Exp Paradigm: Apneas were defined as a pause in the breathing cycle lasting longer than 1.5 seconds. |
|
|
| Developmental profile |
Mortality/lethality1 |
Ameliorated
Description: Dcs treatment increased the survival rate from 13.5 weeks to 18.5 weeks in mecp2 null mice.
Exp Paradigm: NA
|
General observations |
4 months |
| |
|
Description: Dcs treatment increased the survival rate from 13.5 weeks to 18.5 weeks in mecp2 null mice.
Exp Paradigm: NA |
|
|
| Developmental profile |
General characteristics1 |
No adverse effect
Description: Dcs treatment showed no change in coat condition, appearance of eyes, and body stance in mecp2 mutants.
Exp Paradigm: A score for general characteristics was determined by means of a 3 point observational scoring system that indicated coat condition, appearance of eyes, and body stance. a score of 0 meant clean shiny coat, clear eyes and normal stance. a score of 1 indicated dull coat or poorly-groomed appearance, dull eyes, and mildly hunched stance. a score of 2 indicated piloerection, crusted or narrowed eyes, and fully hunched stance.
|
General observations |
4 months |
| |
|
Description: Dcs treatment showed no change in coat condition, appearance of eyes, and body stance in mecp2 mutants.
Exp Paradigm: A score for general characteristics was determined by means of a 3 point observational scoring system that indicated coat condition, appearance of eyes, and body stance. a score of 0 meant clean shiny coat, clear eyes and normal stance. a score of 1 indicated dull coat or poorly-groomed appearance, dull eyes, and mildly hunched stance. a score of 2 indicated piloerection, crusted or narrowed eyes, and fully hunched stance. |
|
|
| Developmental profile |
Size/growth1 |
No adverse effect
Description: Dcs treatment had no adverse effecton the body weight of mecp2 null mice, indicating no obvious toxicity of the drug.
Exp Paradigm: NA
|
Body weight measurement |
1-4 months |
| |
|
Description: Dcs treatment had no adverse effecton the body weight of mecp2 null mice, indicating no obvious toxicity of the drug.
Exp Paradigm: NA |
|
|
|
| Not Reported: |
Circadian sleep/wake cycle, Communications, Emotion, Immune response, Learning & memory, Maternal behavior, Molecular profile, Neuroanatomy / ultrastructure / cytoarchitecture, Physiological parameters, Repetitive behavior, Seizure, Sensory, Social behavior, |
