Analysis Tools
homeostasis/metabolism
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• at 8 months of age, exhibit an even higher level of accumulation of very long chain fatty acids in the spinal cord (C24:0 and C26:0), sciatic nerve (C26:0), adrenal gland (C22:0, C24:0, and C26:0) and serum (C26:0) than seen in single homozygous Abcd1 or Abcd2 mutant mice
(J:94583)
• accumulation of very long chain fatty acids (C26:0) in the dorsal root ganglia
(J:104122)
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nervous system
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• marked increase in the number and size of microglia at 22 months of age
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• marked increase in the number and size of astrocytes at 12 and 22 months of age
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astrocytosis
(
J:94583
)
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• spinal cord axon degeneration that is more severe than in single homozygous Abcd2 or hemizygous Abdc1 mutant mice
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• at 12 months of age, exhibit a lower amplitude of the H-wave, indicating defective spinal somatosensory response/spinal reflex and functionally defective large proprioceptive sensory neurons at the dorsal root ganglia or their afferents
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• the latency of motor waves and the sensitive nerve conduction velocities are slowed down at 12 months of age
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behavior/neurological
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• those mutants with more severe behavioral phenotypes, show exaggerated bilateral extension of fore and hind limbs when suspended by tails at 20 months of age
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• at 15 months of age, exhibit a significant increase in the time required to reach a platform along a balance bar
• at 20 months of age, 6 of 11 double mutants cannot perform the balance test because they freeze or fall repeatedly from the bar
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• severe impairment on the rotarod at 15 months of age
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• at 15 months of age display ventral recumbence, with the entire body flattened against the surface of the balance bar, and the hind and fore limbs wrapped laterally around the bar
• at 20 months of age, see postural hypotonia, characterized by lying flat on abdomen with fore and hind limbs splayed laterally, however muscle strength is no different from wild-type
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• at 15 months of age, do not use the hind limbs when moving along a beam, instead use the fore limbs to drag themselves
• exhibit a decline in deambulation activity at 20 months of age
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• at 15 months of age exhibit a marked tendency to slip off a balance bar, indicating ataxia
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• in the open field, are less explorative than single hemizygous Abcd1 mutant mice and have a tendency to freezing
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• significantly poorer rearing behaviors than wild-type at 20 months of age
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endocrine/exocrine glands
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• accumulation of lipid droplets across the adrenal cortex, encompassing glomerular, fascicular and reticular cell layers
• observe intralysosomal needle-like cholesterol inclusions spread all over the cytoplasm that occur about 10x more often than in hemizygous Abcd1 mutant males
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immune system
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• marked increase in the number and size of microglia at 22 months of age
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• detect some inflammatory cell infiltrates of cervical and lumber spinal cord regions in small clusters adjacent to blood vessels and perimeningeal areas, composed mainly of T lymphocytes
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hematopoietic system
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• marked increase in the number and size of microglia at 22 months of age
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