behavior/neurological

impaired coordination ( J:138147 )

• mild motor deficits detected by an accelerating rotarod test that become prominent at 8 months of age

• performance in an accelerating rotarod test declines until about 12 months of age

abnormal physical strength ( J:138147 )

• modest decrease in performance in a muscle strength test

nervous system

abnormal corticospinal tract morphology ( J:138147 )

• at 18 months of age a high number of degenerating axons are found in corticospinal tracts

abnormal axon morphology ( J:138147 )

• the periaxonal space, of myelinated motor axons in the sciatic nerve and CNS corticospinal tract, is frequently dilated

abnormal ventral spinal root morphology ( J:138147 )

• slight decrease in axon numbers in L5 ventral roots

abnormal axonal transport ( J:138147 )

• transport of tubulin, but not of neurofilaments, is slower compared to controls

axon degeneration ( J:138147 )

• at 18 months of age a high number of degenerating axons are found in corticospinal tracts

Mouse Models of Human Disease		OMIM ID	Ref(s)
NOT	Amyotrophic Lateral Sclerosis 2, Juvenile; ALS2	205100	J:138147