These transgenic mice express elevated levels of SOD1 and develop pathology resembling human Amyotrophic Lateral Sclerosis (ALS). These mice may be useful in studying neuromuscular disorders, including ALS.
Dr. Don Cleveland, Ludwig Institute for Cancer Research (UCSD)
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
Mice hemizygous for this G37R-SOD1 transgene are viable and fertile. The expressed G37R mutant form of human SOD1 is characterized as an enzymatically active, "gain of adverse function" mutation. Hemizygotes develop symptoms and pathology resembling human Amyotrophic Lateral Sclerosis (ALS), with paralyzation in one or more limbs attributable to the loss of motor neurons from the spinal cord. Transgenic mice from the highest expressing founder line (G37R(42) or line 42) express a 14-fold increase in SOD1 activity in spinal cord High expression of G37R-SOD1 is associated with ALS pathology in motor neurons of the spinal cord and brainstem, widespread degenerative changes in other neuronal populations, and mild-to-moderate vacuolar changes in kidney. These high-expressing G37R(42) (or G37R-SOD1 line 42) transgenic mice may be useful in studying neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease).
The original publication by Wong et al assessed survival on a mixed genetic background and noted death occurring around 3.5-4 months of age. Since then, this allele has been backcrossed to C57BL/6J and made fully congenic. This change in genetic background has resulted in a change in disease onset and progression. In a study conducted at The Jackson Laboratory involving a cohort of 21 female transgenic mice, it was found that 90% of the animals survived to 25 weeks, 50% survived to 27 weeks, and death occurred in 100% of the mice by 29 weeks. IN SUMMARY: ON A C57BL/6J BACKGROUND SURVIVAL IS INCREASED TO 6-7 MONTHS.
The G37R-SOD1 (or SOD1-G37R) transgene was designed with a mutant human SOD1 gene (harboring a single amino acid substitution of glycine to arginine at codon 37) driven by its endogenous human promoter. This 12 kb transgene was microinjected into hybrid (C57BL/6J x C3H/HeJ)F2 mouse embryos and transgenic G37R-SOD1 mice (founder line 42) were established. These mice were then backcrossed to C57BL/6J inbred mice for many generations prior to arrival at The Jackson Laboratory.
Expressed Gene | SOD1, superoxide dismutase 1, human |
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Site of Expression |
Allele Name | transgene insertion 42, Donald L Price |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | G37R SOD1 line 42; G37R(42); SOD1 G37R line 42; Tg(SOD1-G37R)42Dpr |
Gene Symbol and Name | Tg(SOD1*G37R)42Dpr, transgene insertion 42, Donald L Price |
Gene Synonym(s) | |
Promoter | SOD1, superoxide dismutase 1, human |
Expressed Gene | SOD1, superoxide dismutase 1, human |
Strain of Origin | (C57BL/6J x C3H/HeJ)F2 |
Chromosome | UN |
Molecular Note | A 12 kb genomic DNA fragment containing a mutated form of human SOD1 with a glycine to arginine substitution at amino acid 37 (G37R) was used for the transgene. The G37R mutation is found in some cases of familial amyotrophic lateral sclerosis (FALS). |
Mutations Made By | Dr. Don Cleveland, Ludwig Institute for Cancer Research (UCSD) |
Mutant mice were bred to C57BL/6J mice to generate this congenic strain. When maintaining the live congenic colony, female wildtype (noncarriers) or C57BL/6J inbred mice can be bred to carrier males. It has been the experience in The Jackson Laboratory Repository colony that hemizygous females do not produce well.
When using the G37R(42) SOD1 mouse strain in a publication, please cite the originating article(s) and include JAX stock #008342 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non carrierfor Tg(SOD1*G37R)42Dpr |
Frozen Mouse Embryo | B6.Cg-Tg(SOD1*G37R)42Dpr/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6.Cg-Tg(SOD1*G37R)42Dpr/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6.Cg-Tg(SOD1*G37R)42Dpr/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | B6.Cg-Tg(SOD1*G37R)42Dpr/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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