Overview

Also Known As: G93A SOD1

These C57BL/6J-congenic SOD1-G93A transgenic mice carry a reduced copy number of the variant human superoxide dismutase 1 soluble gene transgene, Tg(SOD1*G93A)^dl1Gur. These mice exhibit a delayed onset of phenotype when compared to the original high copy number C57BL/6J-congenic SOD1-G93A transgenic mice (B6.Cg-Tg(SOD1*G93A)1Gur/J; Stock No. 004435). These mice are useful for studies of neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS).

Donating Investigator

IMR Colony, The Jackson Laboratory

Genetic overview

Genetic Background	Generation
000664 C57BL/6J

Tg(SOD1*G93A)^dl1Gur

Allele Type
Transgenic (Inserted expressed sequence, Humanized sequence)

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Research Applications

Metabolism Research
Neurobiology Research
Mouse/Human Gene Homologs

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Detailed Description

Mice carrying the transgene exhibit paralysis resulting in a reduced life expectancy. They serve as a model for human amyotrophic lateral sclerosis (ALS). The transgene carries a variant of the human Superoxide dismutase-1 gene (glycine to alanine at position 93). Paralysis is due to loss of motor neurons from the spinal cord. The onset of the ALS phenotype is delayed compared to the original high copy number C57BL/6J-congenic SOD1-G93A transgenic mice (B6.Cg-Tg(SOD1*G93A)1Gur/J; Stock No. 004435) because of a reduction in transgenic copy number (see details below). This reduction most likely occurred during the importation and establishment of the original high copy number mutant transgenic strain. Mice on the B6SJL background (Stock No. 002300) become paralyzed in one or more limbs beginning around six to seven months of age. Life expectancy is normally four to six weeks beyond onset of symptoms. The transgene was backcrossed to C57BL/6J for five generations. The ALS phenotype on this genetic background has not been characterized; however, the onset of the ALS phenotype does not appear to be accelerated (March 1996). One advantage to working with this strain would be its inbred background.

In 2014, Genetic Quality Assurance/Control program at The Jackson Laboratory Repository assessed the survival of a cohort of hemizygous mice (16 females and 9 males; mixed siblings and non-siblings). This showed all tested animals survived beyond 200 days, ~50% survival at 275-300 days of age, and all tested animals die before one year of age. This confirms that reduced transgene copy number in these C57BL/6J-congenic SOD1-G93A transgenic mice (Stock No. 002299) results in a slower progressing model of ALS compared to the original high copy number C57BL/6J-congenic SOD1-G93A transgenic mice (Stock No. 004435; reported to have ~50% survival at 157 days of age).

Development

This transgenic strain was made in the laboratory of Dr. Mark E. Gurney at Northwestern University. The transgene contains the mutant human SOD1 containing the Gly⁹³ -->Ala (G93A) substitution and was microinjected into (C57BL/6 X SJL)F1 fertilized eggs. Upon arrival at The Jackson Laboratory, the mice were crossed to C57BL/6J (Stock No. 000664) for at least five generations.

Expression Data

Expressed Gene	SOD1, superoxide dismutase 1, human
Site of Expression

Control Suggestions

Noncarrier
000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Selected References

Gurney ME; Pu H; Chiu AY; Dal Canto MC; Polchow CY; Alexander DD; Caliendo J; Hentati A; Kwon YW; Deng HX; Chen W; Zhai P; Sufit RL; Siddique T. 1994. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation [see comments] [published erratum appears in Science 1995 Jul 14;269(5221):149] Science 264(5166):1772-5PubMed: 8209258 MGI: J:32665

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Genetics

Tg(SOD1*G93A)^dl1Gur

Allele Symbol: Tg(SOD1*G93A)^dl1Gur

Allele Name	transgene insertion dl 1, Mark E Gurney
Allele Type	Transgenic (Inserted expressed sequence, Humanized sequence)
Allele Synonym(s)	transgene insertion dl 1, Mark E Gurney; Tg(SOD1*G93A)^dl1Gur
Gene Symbol and Name	Tg(SOD1*G93A)^dl1Gur, transgene insertion dl 1, Mark E Gurney
Gene Synonym(s)	G93A SOD1; mSOD1-Tg; Tg(SOD1-G93A)^dl1Gur; TgN(SOD1-G93A)1Gur^dl; TgNSOD1-G93A; TgN(SOD1-G93A)1Gurdl; TgN[SOD1-G93A]^dl1Gur; G1L
Promoter	SOD1, superoxide dismutase 1, human
Expressed Gene	SOD1, superoxide dismutase 1, human
Strain of Origin	(C57BL/6 x SJL)F1
Chromosome	12
General Note	Transgenic mice on a C57BL/6 congenic background exhibit paralysis resulting in a reduced life expectancy, and may serve as a model for human amyotrophic lateral sclerosis (ALS). Paralysis in these mice is due to loss of motor neurons from the spinal cord. The onset of the ALS phenotype in these mice is delayed compared to mice carrying the original high copy number transgene Tg(SOD1*G93A)1Gur. Transgenic mice on a genetic background involving C57BL/6 and SJL become paralyzed in one or more limbs beginning around six to seven months of age. Life expectancy for these mice is normally four to six weeks beyond onset of symptoms.
Molecular Note	This subline has approximately 30% fewer copies of the transgene construct than the high copy number line, Tg(SOD1*G93A)1Gur. The reduction most likely occurred during the importation and establishment of the high copy number mutant transgenic line. The transgene is composed of a variant of the human superoxide dismutase 1 gene (SOD1) carrying a glycine to alanine substitution at position 93 (G93A). The G93A mutation does not alter the activity of the protein.
Mutations Made By	Dr. Mark Gurney, Tetra Discovery Partners

Disease/Phenotype

Disease Terms

Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.

amyotrophic lateral sclerosis type 1

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Metabolism Research
Neurobiology Research
- Metabolic Defects
- Neurodegeneration
- Amyotrophic Lateral Sclerosis (ALS)
Mouse/Human Gene Homologs
- amyotrophic lateral sclerosis (ALS)

Mammalian Phenotype Terms by Genotype

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Tg(SOD1G93A)^dl1Gur/0
involves: C57BL/6 CBA * SJL

behavior/neurological phenotype

impaired coordination
- inability to perform hanging wire test

cellular phenotype

abnormal mitochondrion morphology
- vacuolated mitochondria are sometimes seen in spinal cord of end-stage animals

mortality/aging

premature death
- most symptomatic animals die at <300 days of age

muscle phenotype

muscle weakness
- first sign of disease is forelimb weakness

progressive muscle weakness
- animals show forelimb onset of disease
- the T3 transgene has marginal effects on disease onset and survival in the G1del mutants; onset is 168 to 197 days with end-stage reached at 210-248 days

nervous system phenotype

astrocytosis

Genotype: Tg(SOD1G93A)^dl1Gur/0
involves: C57BL/6 SJL

behavior/neurological phenotype

hindlimb paralysis

limb grasping
- develops at day 200

paralysis
- at day 250

mortality/aging

premature death
- mean 266 day

muscle phenotype

muscle weakness
- develops at day 200

nervous system phenotype

abnormal axonal transport
- at day 200, both fast and slow axonal transport are impaired in the ventral root compared to in wild-type axons

abnormal neuron morphology
- mice develop neurofilament-rich Lewy-body like inclusions in the perikarya and proximal axons of the spinal cord motorneurons at day 180 that increase with age but are not as prominent as in Tg(SOD1*G93A)1Gur mice

Genotype: Tg(SOD1G93A)^dl1Gur/0
involves: C57BL/6 C57BL/6J * SJL/J

hematopoietic system phenotype

microgliosis
- increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age

immune system phenotype

microgliosis
- increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age

nervous system phenotype

astrocytosis
- increased numbers of reactive astrocytes in facial and hypoglossal nuclei at 8 months of age

decreased motor neuron number
- lower numbers of facial and hypoglossal motoneurons at 8 months of age

microgliosis
- increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age

motor neuron degeneration
- atrophic facial and hypoglossal motoneurons at 8 months of age

References

Gurney ME; Pu H; Chiu AY; Dal Canto MC; Polchow CY; Alexander DD; Caliendo J; Hentati A; Kwon YW; Deng HX; Chen W; Zhai P; Sufit RL; Siddique T. 1994. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation [see comments] [published erratum appears in Science 1995 Jul 14;269(5221):149] Science 264(5166):1772-5PubMed: 8209258 MGI: J:32665

Additional References

Alexianu ME; Kozovska M; Appel SH. 2001. Immune reactivity in a mouse model of familial ALS correlates with disease progression. Neurology 57(7):1282-9PubMed: 11591849 MGI: J:78936
Jung C; Rong Y; Doctrow S; Baudry M; Malfroy B; Xu Z. 2001. Synthetic superoxide dismutase/catalase mimetics reduce oxidative stress and prolong survival in a mouse amyotrophic lateral sclerosis model. Neurosci Lett 304(3):157-60PubMed: 11343826 MGI: J:85031
Nagano S; Satoh M; Sumi H; Fujimura H; Tohyama C; Yanagihara T; Sakoda S. 2001. Reduction of metallothioneins promotes the disease expression of familial amyotrophic lateral sclerosis mice in a dose-dependent manner. Eur J Neurosci 13(7):1363-70PubMed: 11298796 MGI: J:85032

Additional - Tg(SOD1*G93A)^dl1Gur related

Technical Support

Contact Technical Support

Genotyping Protocols
- QPCR: Sod TgN Copy Number
- MELT: Tg(SOD)
- Genotyping resources and troubleshooting
Breeding Considerations

When maintaining a live colony, hemizygous mice may be bred to wildtype siblings, or to C57BL/6J inbred mice (Stock No. 000664).
- Additional Breeding and Husbandry Support
Appearance
- black
  Related Genotype: a/a
Citation
When using the G93A SOD1 mouse strain in a publication, please cite the originating article(s) and include JAX stock #002299 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service	Genotype	Price
	Carrier or Non carrier for Tg(SOD1*G93A)

We will fulfill your order by providing at least two carriers for each strain ordered. The total number, sex, and genotypes provided will vary, although typically 8 or more animals are provided. Please check genotypes which will be recovered. While the genotypes of all animals produced will be communicated to you prior to scheduling shipment, the genotypes of animals provided may not reflect the mating scheme and genotypes described in the strain description. Animals are typically ready to ship in 11-14 weeks. If a second recovery is required to produce the minimum number of animals, then delivery time would increase to approximately 25 weeks. If we fail to produce animals of the correct genotype, you will not be charged. We cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation.

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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.

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Also Known As: G93A SOD1

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Tg(SOD1*G93A)dl1Gur

Allele Symbol: Tg(SOD1*G93A)dl1Gur

Disease Terms

Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Mammalian Phenotype Terms by Genotype

Genotype: Tg(SOD1*G93A)dl1Gur/0involves: C57BL/6 * CBA * SJL

behavior/neurological phenotype

cellular phenotype

mortality/aging

muscle phenotype

nervous system phenotype

Genotype: Tg(SOD1*G93A)dl1Gur/0involves: C57BL/6 * SJL

behavior/neurological phenotype

mortality/aging

muscle phenotype

nervous system phenotype

Genotype: Tg(SOD1*G93A)dl1Gur/0involves: C57BL/6 * C57BL/6J * SJL/J

hematopoietic system phenotype

immune system phenotype

nervous system phenotype

References

Additional References

Additional - Tg(SOD1*G93A)dl1Gur related

Genotyping Protocols

Breeding Considerations

Appearance

Citation

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Live Mouse

Cryorecovery - Pricing

Related Products and Services

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms of Use

Additional Use Restrictions Apply

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Tg(SOD1*G93A)^dl1Gur

Allele Symbol: Tg(SOD1*G93A)^dl1Gur

Genotype: Tg(SOD1G93A)^dl1Gur/0
involves: C57BL/6 CBA * SJL

Genotype: Tg(SOD1G93A)^dl1Gur/0
involves: C57BL/6 SJL

Genotype: Tg(SOD1G93A)^dl1Gur/0
involves: C57BL/6 C57BL/6J * SJL/J

Additional - Tg(SOD1*G93A)^dl1Gur related