Analysis Tools
skeleton
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• mice show severe joint contractures by P7
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mortality/aging
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• mice die between 8.5 and 12.5 days of age
(J:131853)
• mice are visibly smaller several days after birth
(J:131853)
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homeostasis/metabolism
growth/size/body
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• mice are visibly smaller several days after birth
• mice weight only 50-60% of controls just before death
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cellular
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• mouse embryonic fibroblasts (MEF) are resistant to apoptosis caused by detachment (anoikis) with 25-30% more viable cells present than wild-type MEFs after 24 hours of suspension culture
• MEFs are more sensitive than wild-type cells to treatment with apoptosis-inducing kinase inhibitor staurosporine
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immune system
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• mice have an 80% reduction of neutrophils in their blood
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behavior/neurological
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• by P7, mice are unable to right themselves
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hematopoietic system
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• mice have an 80% reduction of neutrophils in their blood
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muscle
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• Evans blue dye uptake shows multiple damaged myofibers in the gastrocnemius muscle, indicating sarcolemmal defects
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• in triceps, tibialis anterior and gastrocnemius skeletal muscle
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• the diameter of the myofibers in the epaxial muscle are significantly smaller than in wild-type controls
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• mice show multiple degenerating and regenerating muscle fibers at P7
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• severe myopathic changes typical of muscular dystrophies are seen in skeletal muscle, including centrally nucleated fibers, elevated creatine kinase activity, increased fiber size variation and endomysial fibrosis
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• mice become progressively weaker after birth until death between 8.5 and 12.5 days of age
(J:131853)
• mice become progressively weaker within the first week of life and by P7, they are unable to right themselves, show severe joint contractures and can no longer walk
(J:241883)
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• severe myopathic changes are seen in P7 skeletal muscle, including centrally nucleated fibers in triceps, tibialis anterior and gastrocnemius skeletal muscle, elevated creatine kinase activity, increased fiber size variation, and endomysial fibrosis
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renal/urinary system
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• the subcapsular glomeruli are smaller than in controls in one week old pups
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