Phenotypes Associated with This Genotype

Genotype
MGI:5644692

• Allelic Composition: Meox2^tm1(cre)Sor/Meox2⁺; Tg(CAG-lacZ,-FUS,-EGFP)629Gyu/0
• Genetic Background: involves: 129S4/SvJaeSor * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

Tg(CAG-lacZ,-FUS,-EGFP)629Gyu/0 Meox2^tm1(cre)Sor/Meox2⁺ mice display hindlimb curl

mortality/aging

premature death ( J:216672 )

• nearly 100% of mice die before P30

growth/size/body

decreased body weight ( J:216672 )

• body weights are different from controls starting at P4

behavior/neurological

impaired righting response ( J:216672 )

• seen by P14

limb grasping ( J:216672 )

• seen at P14

decreased grip strength ( J:216672 )

• seen by P14

abnormal gait ( J:216672 )

• mice develop gait abnormalities at P10

muscle

muscular atrophy ( J:216672 )

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

nervous system

microgliosis ( J:216672 )

• 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

astrocytosis ( J:216672 )

• mice that die by P30 exhibit activation of astrocytes in the brain and spinal cord

abnormal neuromuscular synapse morphology ( J:216672 )

• end-stage mutants show abnormalities and degeneration of the neuromuscular junctions

hematopoietic system

microgliosis ( J:216672 )

• 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

microgliosis ( J:216672 )

• 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