hematopoietic system
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• 9.3% of total blood derived cells express B220+ and no expression of CD19+ cells as determined by cytofluorimetric analysis
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• no expression of CD4+ cells as determined by cytofluorimetric analysis
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• no expression of CD8+ cells as determined by cytofluorimetric analysis
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homeostasis/metabolism
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• mice exhibit a shorter time to exhaustion than wild type controls
• higher endurance on treadmill in double mutant than Dmdmdx mutants
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• costal diaphragm (DIA) has a lower quantity of active TGFB1 in comparison to Dmdmdx mutant although total quantity is similar
• tibialis anterior (TA) and quadricepts (QA) muscles have a lower quantity of total TGFB1 in comparison to Dmdmdx mutant
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immune system
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• 9.3% of total blood derived cells express B220+ and no expression of CD19+ cells as determined by cytofluorimetric analysis
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• no expression of CD4+ cells as determined by cytofluorimetric analysis
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• no expression of CD8+ cells as determined by cytofluorimetric analysis
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muscle
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• area of muscle fibers is 1500-1800 um2 and coefficient of variance is 55-65
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• small, centrally nucleated, regenerating muscle fibers are found in 2 and 12 month old mice
• 46-52% of muscle fibers exhibit centrally located nuclei
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• degenerating muscle fibers are found in 2 and 12 month old mice
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• aged 12 month old double mutant mice exhibit fibrosis, but less than in age-matched Dmdmdx mice
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• loss of normalized tetanic force in the costal diaphragm (DIA) strips is observed in 2 and 12 month old mice as compared to wild type
• change appears lower in double mutant than in Dmdmdx mutants
• a similar decrease in normalized tetanic force occurs in the tibialis anterior (TA)
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reproductive system
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• double mutant mice are less fertile than Dmdmdx mice
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skeleton
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• progressive spinal deformity
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behavior/neurological
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• mice exhibit a shorter time to exhaustion than wild type controls
• higher endurance on treadmill in double mutant than Dmdmdx mutants
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