About   Help   FAQ
Phenotypes Associated with This Genotype
Genotype
MGI:5644692
Allelic
Composition
Meox2tm1(cre)Sor/Meox2+
Tg(CAG-lacZ,-FUS,-EGFP)629Gyu/0
Genetic
Background
involves: 129S4/SvJaeSor * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Meox2tm1(cre)Sor mutation (3 available); any Meox2 mutation (5 available)
Tg(CAG-lacZ,-FUS,-EGFP)629Gyu mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Tg(CAG-lacZ,-FUS,-EGFP)629Gyu/0 Meox2tm1(cre)Sor/Meox2+ mice display hindlimb curl

mortality/aging
• nearly 100% of mice die before P30

growth/size/body
• body weights are different from controls starting at P4

behavior/neurological
• seen at P14
• seen by P14
• mice develop gait abnormalities at P10

muscle
• end-stage mutants exhibit scattered and grouped atrophic muscle fibers and presence of pyknotic myofibers indicating muscle atrophy

nervous system
• mice that die by P30 exhibit activation of microglia in the brain and spinal cord
• mice, however do not exhibit degeneration of axons in the dorsal corticospinal tract or lateral columns, or in the dorsal or ventral roots, indicating that descending motor axons are not altered, show no motor neuron loss in the cervical spinal cord, and no FUS protein aggregates in the brain or spinal cords
• mice that die by P30 exhibit activation of astrocytes in the brain and spinal cord
• end-stage mutants show abnormalities and degeneration of the neuromuscular junctions

hematopoietic system
• mice that die by P30 exhibit activation of microglia in the brain and spinal cord
• mice, however do not exhibit degeneration of axons in the dorsal corticospinal tract or lateral columns, or in the dorsal or ventral roots, indicating that descending motor axons are not altered, show no motor neuron loss in the cervical spinal cord, and no FUS protein aggregates in the brain or spinal cords

immune system
• mice that die by P30 exhibit activation of microglia in the brain and spinal cord
• mice, however do not exhibit degeneration of axons in the dorsal corticospinal tract or lateral columns, or in the dorsal or ventral roots, indicating that descending motor axons are not altered, show no motor neuron loss in the cervical spinal cord, and no FUS protein aggregates in the brain or spinal cords

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 6 DOID:0060198 OMIM:608030
J:216672


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 & Copyright Notice
Send questions and comments to User Support.
last database update
08/04/2020
MGI 6.15
The Jackson Laboratory