Phenotypes Associated with This Genotype

Genotype
MGI:3807481

• Allelic Composition: Als2^tm1Garo/Als2^tm1Garo
• Genetic Background: either: (involves: 129S1/Sv * 129X1/SvJ) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6)

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

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

axon degeneration ( J:138147 )

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

abnormal axonal transport ( J:138147 )

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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
NOT	amyotrophic lateral sclerosis type 2	DOID:0060194	OMIM:205100	J:138147