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Phenotypes Associated with This Genotype
Genotype
MGI:5513111
Allelic
Composition
Mnx1tm4(cre)Tmj/Mnx1+
Tardbptm1.1Ckjs/Tardbptm1.2Cjks
Genetic
Background
involves: 129S1/Sv * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mnx1tm4(cre)Tmj mutation (2 available); any Mnx1 mutation (9 available)
Tardbptm1.1Ckjs mutation (0 available); any Tardbp mutation (25 available)
Tardbptm1.2Cjks mutation (0 available); any Tardbp mutation (25 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• average lifespan of mutants showing amyotrophic lateral sclerosis-like phenotypes is 10 months (J:190254)
• average lifespan of mutants showing amyotrophic lateral sclerosis-like phenotypes is 10 months (J:190254)

growth/size/body
• average weight is slightly lower at early birth than in controls and this difference becomes more pronounced with age, with a significant weight difference after 8 weeks of age (J:190254)
• average weight is slightly lower at early birth than in controls and this difference becomes more pronounced with age, with a significant weight difference after 8 weeks of age (J:190254)
• while mutants show a peak of weight gain during 90-100 days of age, soon after this time, they begin to show weight loss (J:190254)
• while mutants show a peak of weight gain during 90-100 days of age, soon after this time, they begin to show weight loss (J:190254)

behavior/neurological
• abnormal hind limb clasping is seen after 13 weeks of age (J:190254)
• abnormal hind limb clasping is seen after 13 weeks of age (J:190254)
• mutants show a deficiency in the rotarod test after 13 weeks of age (J:190254)
• mutants show a deficiency in the rotarod test after 13 weeks of age (J:190254)

immune system
• microglia activation is seen in the lateroventral lumbar spinal cord (J:190254)
• microglia activation is seen in the lateroventral lumbar spinal cord (J:190254)

muscle
• more males than females develop amyotrophic lateral sclerosis-like phenotypes with a male/female ratio of 3:1 (J:190254)
• more males than females develop amyotrophic lateral sclerosis-like phenotypes with a male/female ratio of 3:1 (J:190254)

nervous system
• microglia activation is seen in the lateroventral lumbar spinal cord (J:190254)
• microglia activation is seen in the lateroventral lumbar spinal cord (J:190254)
• in the spinal cord (J:190254)
• in the spinal cord (J:190254)
• accumulation of ubiquitinated proteins in the spinal cord motor neurons at 20 weeks of age (J:190254)
• accumulation of ubiquitinated proteins in the spinal cord motor neurons at 20 weeks of age (J:190254)
• progressive loss of motor neurons, with a 10% decrease of spinal cord motor neurons at 10 weeks of age and a large loss of ChAT-positive motor neurons at 20 weeks of age (J:190254)
• 46% and 25% reduction in alpha and gamma motor neurons, respectively, in the lumbar regions of the spinal cord at 20 weeks of age (J:190254)
• more males than females develop amyotrophic lateral sclerosis-like phenotypes with a male/female ratio of 3:1 (J:190254)
• progressive loss of motor neurons, with a 10% decrease of spinal cord motor neurons at 10 weeks of age and a large loss of ChAT-positive motor neurons at 20 weeks of age (J:190254)
• 46% and 25% reduction in alpha and gamma motor neurons, respectively, in the lumbar regions of the spinal cord at 20 weeks of age (J:190254)
• more males than females develop amyotrophic lateral sclerosis-like phenotypes with a male/female ratio of 3:1 (J:190254)
• motor neuron loss, reactive astrocytosis, microglia activation and accumulation of polyubiquitinated proteins in the ventral horn (J:190254)
• motor neuron loss, reactive astrocytosis, microglia activation and accumulation of polyubiquitinated proteins in the ventral horn (J:190254)

skeleton
• mutants exhibit kyphosis beginning at 20 weeks of age which becomes severe at 24 weeks (J:190254)
• mutants exhibit kyphosis beginning at 20 weeks of age which becomes severe at 24 weeks (J:190254)

hematopoietic system
• microglia activation is seen in the lateroventral lumbar spinal cord (J:190254)
• microglia activation is seen in the lateroventral lumbar spinal cord (J:190254)

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:190254


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory