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construct
M_MECP2_1.3_KO_HE_ALC
Model Details
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Phenotypic Profile
Category Entity Quantity Experimental Paradigm Age at Testing
Motor phenotype Grip strength1
Ameliorated
Description: Decreased grip strength is partially restored to the level of wild type on pn 22 mice; decreased grip strength is refractoryto the treatment on pn 30 or pn 43 mice
Exp Paradigm: Grip strength assay

Grip strength test 3, 4, 6 weeks
Motor phenotype Grip strength1
Ameliorated
Description: Decreased forepaw grip strength is partially restored to the level of wild type
Exp Paradigm: Grip strength assay

Grip strength test 6 weeks
Motor phenotype General locomotor activity1
Ameliorated
Description: Decreased locomotor activity is partially restored to the level of wild type on pn 21 or pn 29 mice but is refractoryto the treatment on pn 42 mice
Exp Paradigm: General motor activity levels during dark cycle

Light-dark exploration test 3, 4, 6 weeks
Motor phenotype Motor coordination and balance1
Refractory
Description: Decreased motor coordination is refractoryto the treatment
Exp Paradigm: Rotorod test

Accelerating rotarod test 6 weeks
Neuroanatomy / Ultrastructure / Cytoarchitecture Dendritic architecture: spine density1
Restored
Description: Dendritic cell morphology assessed by cell body size, dendritic length and dendritic structure is restored to the same level as wild type
Exp Paradigm: Sholl analysis of cell morphology in the dentate gyrus of the hippocampus

Sholl analysis 6.5 weeks
Neurophysiology Neurotransmitter release: neurotrophins1
Refractory
Description: Decreased bdnf expression in cerebellum or decreased ngf expression in striatum is refractoryto the treatment; there are no adverse effectin bdnf/ngf expresson in other checked brain areas (cortex, hippocampus, and striatum/cerebellum)
Exp Paradigm: Bdnf/ngf elisa's

Immunohistochemistry 6 weeks
Physiological parameters Metabolic function1
Restored
Description: Metabolic function as assessed by measuring carbon dioxide (co2) production and oxygen (o2) consumption is restored to the same level as wild type on pn 43 mice; there is no adverse effectin pn 22 or pn 30 mice
Exp Paradigm: Gas exchange measurements

Measurement of gas exchange 3, 4, 6 weeks
Physiological parameters Bioactive compound levels: carnitine1
Side effect
Description: Increased levels of l-carnitine, alc, and propionyl carnitine (plc) in blood plasma is a direct effect after the treatment
Exp Paradigm: Carnitines levels

High-performance liquid chromatography (hplc) 6.5 weeks
Developmental profile Size/growth1
Ameliorated
Description: Decreased rate of weight gain is restored to the same level as wild type during pn 22-35 but is refractoryto the treatment after pn 36
Exp Paradigm: General observations

General observations 3-5 weeks
Learning & memory Cued or contextual fear conditioning1
Refractory
Description: Decreased in cued conditioning learning is refractoryto the treatment but there is no adverse effectin contextual conditioning learning
Exp Paradigm: Associative fear conditioning task

Fear conditioning test 6 weeks
Learning & memory Object recognition memory1
Ameliorated
Description: Decreased long term memory is partially restored to the level of wild type
Exp Paradigm: Object recognition task

Novel object recognition test 4 weeks
Not Reported: Circadian sleep/wake cycle,  Communications,  Emotion,  Immune response,  Maternal behavior,  Physiological parameters,  Repetitive behavior,  Seizure,  Sensory,  Social behavior,  
 
References: 1:  Schaevitz LR , et al. 2012
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