Analysis Tools
mortality/aging
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• survival rate is decreased by 36%
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• survival rate is decreased by 36%
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behavior/neurological
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• at 4 months, mice exhibit decreased latency to fall on a rotarod compared with wild-type mice
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• towards the end of their lives
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• late in life mice exhibit a shuffle gait with dragging hindlimbs
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nervous system
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• ferritin nuclear inclusions are observed in Purkinje cells and granule cell layer neurons unlike in wild-type mice
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• ferritin nuclear inclusions are observed in Purkinje cells and granule cell layer neurons unlike in wild-type mice
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• mice exhibit an age-dependent increase in the size of ferritin nuclear inclusions and the number of cells with nuclear inclusions compared to in wild-type mice
• mice exhibit ferritin inclusions in the substantia nigra, striatum, amygdala, cerebral cortex, and Purkinje cells and granule cells of the cerebellum unlike in wild-type mice
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homeostasis/metabolism
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• ferritin inclusions are observed in neural tissues; cardiac cells; striated muscle of the tongue; mucosa, muscularis mucosae, submucosa and muscularis external layers of the gastrointestinal tract; hepatocytes of the liver; adrenal glands and adjacent adipose tissue; cuboidal cells of the convolute tubules of the kidneys; connective tissue of the urinary bladder; ureters; cuboidal cells of the excretory ducts and fibroblasts of the reticular layer of the skin; mononuclear cells of lymphoid nodules and endothelial cells of blood vessels of the spleen; striated and smooth muscle tissues; epithelium of uterine glands in females; seminiferous tubes of the testis, intratesticular genitals ducts and epididymidis in males; and alveolar epithelial cells and smooth muscle cells of the lungs
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skeleton
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• mice exhibit a very low body position with some degree of lordosis unlike wild-type mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| neurodegeneration with brain iron accumulation 3 | DOID:0110737 |
OMIM:606159 |
J:131053 | |
