These mice express a transgene containing a missense mutation of the Sod1 gene that confers a progressive loss of motor function.
Dr. Jon W. Gordon, Mount Sinai School of Medicine
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
These transgenic mice express the missense mutant mouse Sod1 under the control of the endogenous promoter. The missense mutation is a point mutation in exon 4 resulting in a glycine-86 to arginine substitution,which corresponds to amino acid position 86 in the human SOD1 protein. Transgene expression is detected by RT-PCR analysis of brain, spinal cord, and other tissues. Onset of progressive loss of motor function begins at 3-4 months of age with hind limb spastic paralysis and muscle wasting. Transgenic mice do not survive beyond 4 months of age. Histological analysis of spinal cord ventral horns, brain stem and neocortex reveals neurodegeneration and abnormal neurites. Affected mice do not exhibit diminished SOD1 activity. This mutant mouse strain may be useful in studies of amyotrophic lateral sclerosis.
A transgenic construct containing 1.5kb sequence of the modified mouse Sod1 gene with the missense mutation in exon 4, and flanking regions was injected into fertilized FVB/N mouse eggs. Founder animals were bred to wildtype FVB/N mice.
Expressed Gene | Sod1, superoxide dismutase 1, soluble, mouse, laboratory |
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Site of Expression |
Allele Name | transgene insertion M1, Jon W Gordon |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | M1; Tg(Sod1*G85R)M1Jwg; Tg(Sod1-G86R)M1Jwg |
Gene Symbol and Name | Tg(Sod1*G86R)M1Jwg, transgene insertion M1, Jon W Gordon |
Gene Synonym(s) | |
Promoter | Sod1, superoxide dismutase 1, soluble, mouse, laboratory |
Expressed Gene | Sod1, superoxide dismutase 1, soluble, mouse, laboratory |
Strain of Origin | FVB/N |
Chromosome | UN |
Molecular Note | The entire mouse Sod1 gene with an engineered point mutation in exon 4 resulting in a glycine to arginine substitution at residue 86 (G86R) was used as the transgene. Mutation G86R corresponds to the SOD1 G86R mutation found in some cases of human amyotrophic lateral sclerosis (ALS) patients. RT-PCR analysis detected expression of the transgene in brain, spinal cord, and other tissues. The missense mutation is a point mutation in exon 4 resulting in a glycine-86 to arginine substitution, which corresponds to amino acid position 86 in the human SOD1 protein. Transgenics exhibit widespread high expression of the transgene and do not exhibit diminished SOD1 activity. . |
Mutations Made By | Dr. Jon Gordon, Mount Sinai School of Medicine |
This strain is maintained as a hemizgyote. The Donating Investigator reports that female transgenic mice have reduced fertility, small litters and poor milk production.
When using the FVB-Tg(Sod1*G86R)M1Jwg/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #005110 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non carrier for Tg(Sod1*G86R)M1Jwg |
Frozen Mouse Embryo | FVB-Tg(Sod1*G86R)M1Jwg/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | FVB-Tg(Sod1*G86R)M1Jwg/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | FVB-Tg(Sod1*G86R)M1Jwg/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | FVB-Tg(Sod1*G86R)M1Jwg/J Frozen Embryo | $3373.50 |
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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.
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