Analysis Tools
homeostasis/metabolism
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• at 8 months of age, exhibit an accumulation of very long chain fatty acids in the spinal cord (C26:0), adrenal gland (C24:0 and C26:0), and sciatic nerve (C26:0)
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behavior/neurological
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• in rotarod test at 20 months of age
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• impaired exploratory abilities in the open field test
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endocrine/exocrine glands
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• exhibit intralysosomal needle-like cholesterol inclusions in the fascicular cell layer of the cortex and cortico-medullary junction
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• at 18 months of age, mice exhibit fibrosis of the fascicular cell layer
• mice exhibit intralysosomal needle-like cholesterol inclusions in the fascicular cell layer
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• at 18 months of age, exhibit fibrosis of the reticular cell layer
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nervous system
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• slight increase in the number and size of microglia at 22 months of age
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• slight increase in the number and size of astrocytes at 22 months of age
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astrocytosis
(
J:94583
)
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• axonal degeneration in the spinal cord and inflammatory infiltrates in cervical and lumbar spinal cord regions
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• degeneration in the spinal cord, characterized by axonal swelling with accumulation of APP and synaptophysin and abnormal lipidic droplets resulting from myelin debris
• axonal degeneration precedes myelin disturbances
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• spinal cord axons contain myelin debris
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• exhibit slowing of motor and sensitive nerve conduction velocities at 20, but not 12, months of age
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hematopoietic system
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• slight increase in the number and size of microglia at 22 months of age
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immune system
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• slight increase in the number and size of microglia at 22 months of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| adrenoleukodystrophy | DOID:10588 |
OMIM:300100 |
J:94583 | |
