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Phenotypes Associated with This Genotype
Genotype
MGI:5544197
Allelic
Composition
Tg(Thy1-VAPB*P56S,-EGFP)D3Cai/0
Genetic
Background
C57BL/6-Tg(Thy1-VAPB*P56S,-EGFP)D3Cai
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• motor coordination and balance are impaired on the rotarod beginning at 12 months of age
• however, grip strength is normal
• mice start to exhibit hyperactivity in vertical movements in the open-field test at 12 months of age
• mice start to exhibit hyperactivity in horizontal movements in the open-field test at 12 months of age
• stride length and stride time are shorter, first detected at 2 months of age and the gait abnormality is persistent without deterioration throughout life

growth/size/body
• body weight is less starting at 15 months of age

nervous system
• the number of corticospinal motor neurons is decreased at 10 and 18 months of age, but not at 3 months of age
• severe reduction of corticospinal track in the thoracic spinal cord at 12 months of age
• endoplastic reticulum morphology in cortical neurons is altered, showing large convoluted membranous stacks rather than the normal small individual punctate ones
• the number of corticospinal motor neurons is decreased at 10 and 18 months of age, but not at 3 months of age
• more than 60% of CTIP2-positive corticospinal motor neurons are lost in the motor cortex of 18 month old mice
• 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
• mice develop progressive degeneration of corticospinal motor neurons but not spinal motor neurons
• the length of the longest axis of C-boutons of spinal motor neurons is increased
• 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

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


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory