behavior/neurological
• paralysis in one or both hind limbs begins at approximately 4 months of age, with 54.1% of mice showing paralysis by 5 months of age
|
growth/size/body
weight loss
(
J:234172
)
homeostasis/metabolism
dehydration
(
J:234172
)
immune system
dermatitis
(
J:234172
)
integument
dermatitis
(
J:234172
)
neoplasm
• 79.2% of mice exhibit neurofibroma tumors; tumors arise at the dorsal root ganglia, with invasion of the neural foramen and lead to compression of the spinal cord
• most tumors are detected in cervical and thoracic spine, with a lower percentage in the lumbar spine
• neurofibromas are characterized by disorganized Remak bundles and enriched deposition of collagen
|
nervous system
• 79.2% of mice exhibit neurofibroma tumors; tumors arise at the dorsal root ganglia, with invasion of the neural foramen and lead to compression of the spinal cord
• most tumors are detected in cervical and thoracic spine, with a lower percentage in the lumbar spine
• neurofibromas are characterized by disorganized Remak bundles and enriched deposition of collagen
|
• enlarged peripheral nerves
|
• enlarged spinal nerve roots and lesions in sciatic nerves showing abnormal nonmyelinating axons
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
neurofibromatosis 1 | DOID:0111253 |
OMIM:162200 |
J:234172 |