Mouse Genome Informatics
hm
    Als2tm1Garo/Als2tm1Garo
either: (involves: 129S1/Sv * 129X1/SvJ) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6)
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• 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
• modest decrease in performance in a muscle strength test

nervous system
• at 18 months of age a high number of degenerating axons are found in corticospinal tracts
• the periaxonal space, of myelinated motor axons in the sciatic nerve and CNS corticospinal tract, is frequently dilated
• slight decrease in axon numbers in L5 ventral roots
• transport of tubulin, but not of neurofilaments, is slower compared to controls
• at 18 months of age a high number of degenerating axons are found in corticospinal tracts

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