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Phenotypes Associated with This Genotype
Genotype
MGI:3616467
Allelic
Composition
Als2tm1Jei/Als2tm1Jei
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Als2tm1Jei mutation (0 available); any Als2 mutation (51 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• between 7 and 18 months of age, there is a progressive enhancement of inflammation in the brain and spinal cord
• at 18 months of age, there is a 30.9% reduction in Purkinje cell soma size compared to wild-type
• at 18 months of age, a 22.9% reduction in Purkinje cell number is detected compared to wild-type
• between 7 and 18 months of age, there is a progressive enhancement of astrocytosis in the brain and spinal cord
• at 12 months, the number of viable axons (motor units) is significantly reduced in the hindlimb
• decrease in axon numbers and concurrent decrease in single motor units suggests cycles of motorneuron degeneration followed by reinnervation
• at 18 months, the number of motor axons in the L4 ventral spinal cord is significantly reduced
• at 12 and 20 months, there are fewer neuromuscular junctions detected in the gastrocnemious muscle
• at 20 months, postsynaptic folding in the neuromuscular junction is less complex, smaller, and more globular in conformation with respect to the wild-type
• at 12 months, single motor unit potential amplitude is significantly larger in homozygous mice

behavior/neurological
N
• no signs of motor abnormalities are seen in mice up to 21 months of age

muscle
• the thoracic muscle shows regions of dense pyknotic nuclear clumping
• the thoracic muscle shows isolated angular atrophic muscle fibers
• the thoracic muscle shows central nuclei
• at 20 months of age in the gastrocnemius muscle, the pattern of fiber distribution is severly abnormal

immune system
• between 7 and 18 months of age, there is a progressive enhancement of inflammation in the brain and spinal cord

Mouse Models of Human Disease
OMIM ID Ref(s)
NOT Amyotrophic Lateral Sclerosis 2, Juvenile; ALS2 205100 J:104446


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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
07/19/2016
MGI 6.04
The Jackson Laboratory