Mouse Genome Informatics
tg
    Tg(Thy1-VAPB*P56S,-EGFP)D3Cai/0
C57BL/6-Tg(Thy1-VAPB*P56S,-EGFP)D3Cai
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• motor coordination and balance are impaired on the rotarod beginning at 12 months of age (J:202148)
• however, grip strength is normal (J:202148)
• mice start to exhibit hyperactivity in vertical movements in the open-field test at 12 months of age (J:202148)
• mice start to exhibit hyperactivity in horizontal movements in the open-field test at 12 months of age (J:202148)
• stride length and stride time are shorter, first detected at 2 months of age and the gait abnormality is persistent without deterioration throughout life (J:202148)

growth/size/body
• body weight is less starting at 15 months of age (J:202148)

nervous system
• the number of corticospinal motor neurons is decreased at 10 and 18 months of age, but not at 3 months of age (J:202148)
• severe reduction of corticospinal track in the thoracic spinal cord at 12 months of age (J:202148)
• endoplastic reticulum morphology in cortical neurons is altered, showing large convoluted membranous stacks rather than the normal small individual punctate ones (J:202148)
• the number of corticospinal motor neurons is decreased at 10 and 18 months of age, but not at 3 months of age (J:202148)
• more than 60% of CTIP2-positive corticospinal motor neurons are lost in the motor cortex of 18 month old mice (J:202148)
• loss of corticospinal motor neurons in the cerebral cortex is selective, with no degeneration in layer V pyramidal neurons of the posterior cerebral cortex or in striatal medium spiny neurons (J:202148)
• mice develop progressive degeneration of corticospinal motor neurons but not spinal motor neurons (J:202148)
• the length of the longest axis of C-boutons of spinal motor neurons is increased (J:202148)
• intensity of L5 ventral root discharges at all stimulus levels are decreased in spinal cords of P10-P11 mice and the intensity of spontaneous rhythmic discharges in disinhibited spinal cords is reduced, indicating that C-bouton-mediated muscarinic receptor function is compromised (J:202148)

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 8; ALS8 608627 J:202148