nervous system
• increased numbers of microglia accompany degenerative changes
|
microgliosis
(
J:245100
)
• microgliosis in the hippocampal fimbria at 26 weeks of age
|
• hippocampal fimbria exhibits APP+ axonal spheroids, microgliosis and astrogliosis
|
• mild astrocytosis accompanies degenerative changes
(J:48031)
• moderate, but significant, astrogliosis in the hippocampal fimbria at 26 weeks of age
(J:245100)
|
• occasionally inner tongue processes of pligdendrocytes contain degenerated organelles
(J:48031)
• primary glial cultures have increased numbers of oligodendrocytes starting at the second division and persisting through at least the fourteenth division; however no increase in oligodendrocyte proliferation is seen
(J:106182)
• in primary glial cultures oligodendrocyte sheets appear larger, vacuolated, and broken suggesting decreased stability of the myelin sheet
(J:106182)
|
• at 6 - 8 weeks, focal axonal swellings containing organelles are seen mostly in areas where small diameter axons predominate throughout the white and gray matter
• by 1 year, numerous large axonal swellings are seen in the optic nerve and spinal cord with some also seen in Purkinje cell axons
• after 1 year, axonal degeneration is seen in the optic nerve and fasciculus gracilis; however, no signs of peripheral neuropathy are seen
|
• in areas of axonal swelling the myelin sheath becomes attenuated and is eventually lost though slippage
(J:48031)
• decrease in cholesterol content
(J:193310)
|
• APP+ axonal spheroids are present in the hippocampal fimbria and corpus callosum at 26 weeks of age
|
demyelination
(
J:48031
)
• evidence of demyelination is seen at 22 months of age
|
behavior/neurological
• at 16 months of age impaired performance in a rotarod test is seen
|
• at 16 months of age impaired performance in a rotarod test is seen
|
abnormal gait
(
J:48031
)
• older mice develop a slow gait
|
hematopoietic system
• increased numbers of microglia accompany degenerative changes
|
microgliosis
(
J:245100
)
• microgliosis in the hippocampal fimbria at 26 weeks of age
|
immune system
• increased numbers of microglia accompany degenerative changes
|
microgliosis
(
J:245100
)
• microgliosis in the hippocampal fimbria at 26 weeks of age
|
homeostasis/metabolism
• in myelin
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
hereditary spastic paraplegia 2 | DOID:0110773 |
OMIM:312920 |
J:48031 , J:245100 |