About   Help   FAQ
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 (29 available)
Tardbptm1.1Ckjs mutation (0 available); any Tardbp mutation (68 available)
Tardbptm1.2Cjks mutation (0 available); any Tardbp mutation (68 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

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
• while mutants show a peak of weight gain during 90-100 days of age, soon after this time, they begin to show weight loss

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

immune system
• microglia activation is seen in the lateroventral lumbar spinal cord

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

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

skeleton
• mutants exhibit kyphosis beginning at 20 weeks of age which becomes severe at 24 weeks

hematopoietic system
• microglia activation is seen in the lateroventral lumbar spinal cord

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 10 DOID:0060201 OMIM:612069
J:190254


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
11/12/2024
MGI 6.24
The Jackson Laboratory