Strain Name:

B6.Cg-Tg(SOD1*G37R)1Dwc/J

Stock Number:

016149

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Availability:

Cryopreserved - Ready for recovery

These LoxSOD1G37R mice harbor a floxed transgene comprised of the human SOD1 gene bearing a G37R mutation that is causative of inherited Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease). These mice develop ALS-like fatal, progressive paralysis by 1 year of age.

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Generation+pN1
Generation Definitions
 
Donating InvestigatorDr. Don Cleveland,   Ludwig Institute for Cancer Res. (UCSD)

Description
The LoxSOD1G37R transgene has loxP sites flanking a human G37R mutant superoxide dismutase 1, soluble gene sequence (SOD1*G37R) that is characterized as an enzymatically active, "gain of adverse function" mutation. SOD1 is a widely expressed isozyme responsible for destroying free superoxide radicals. SOD1 mutations, including SOD1*G37R, are known to cause Amyotrophic Lateral Sclerosis (ALS). Hemizygotes mice are viable and fertile. These mice develop symptoms and pathology resembling human ALS, including progressive weight loss from denervation-induced muscle atrophy and paralyzation. Transgenic mice develop the fatal progressive motor neuron disease and die by 12-14 months of age, with neuron death in 55% of spinal motor neurons.
When LoxSOD1G37R transgenic mice are bred to mice expressing tissue-specific Cre recombinase, the resulting offspring will have the floxed-SOD1*G37R transgene deleted in the cre-expressing tissues. When SOD1*G37R expression is deleted in microglia and myeloid cells, the progression of disease was delayed. When SOD1*G37R expression is deleted in Schwann cells, disease progression was significantly accelerated. These mice may be useful in studying SOD1 in neuromuscular disorders, including ALS or Lou Gehrig's Disease.

Development
The SOD1*G37R transgene was designed with the entire human floxed-superoxide dismutase 1, soluble (SOD1) gene, modified to harbor the SOD1*G37R mutation, driven by its endogenous human promoter. The transgene was microinjected into fertilized (C57BL/6J x C3H/HeJ) F2 oocytes. SOD1*G37R mice from founder line 1 were backcrossed onto a C57BL/6 background for at least 30 generations (see SNP note below). Upon arrival at The Jackson Laboratory Repository, mice were bred to C57BL/6J inbred mice (Stock No. 000664) for at least one generation to establish the colony.

A 32 SNP (single nucleotide polymorphism) panel analysis, with 27 markers covering all 19 chromosomes and the X chromosome, as well as 5 markers that distinguish between the C57BL/6J and C57BL/6N substrains, was performed on the rederived living colony at The Jackson Laboratory Repository. While the 27 markers throughout the genome suggested a C57BL/6 genetic background, all 5 markers that determine C57BL/6J from C57BL/6N were found to be segregating. These data suggest the mice sent to The Jackson Laboratory Repository were on a C57BL/6N genetic background.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J (approximate)
   005304 C57BL/6NJ (approximate)
 
  Considerations for Choosing Controls

Related Strains

View Amyotrophic Lateral Sclerosis (ALS)     (30 strains)

View Strains carrying other alleles of SOD1     (15 strains)

Additional Web Information

Working with ALS Mice manual [.pdf]
This resource was prepared by scientists with Prize4Life and The Jackson Laboratory.

Introduction to Cre-lox technology

Visit the Amyotrophic Lateral Sclerosis (ALS) Mouse Model Resource site for helpful information on ALS Disease and research resources.

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on transgenic expression of an ortholog of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Amyotrophic Lateral Sclerosis 1; ALS1   (SOD1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Tg(SOD1*G37R)1Dwc/0

        involves: C57BL/6
  • mortality/aging
  • premature death
    • transgenic mice develop end-stage disease by 8.5-11 months of age   (MGI Ref ID J:109131)
  • growth/size/body phenotype
  • weight loss
    • mice display weight loss from denervation induced muscle atrophy   (MGI Ref ID J:109131)
  • nervous system phenotype
  • decreased motor neuron number
    • mice in end-stage disease display 55% spinal motor neuron death   (MGI Ref ID J:109131)
  • motor neuron degeneration
    • mice develop fatal progressive motorneuron disease   (MGI Ref ID J:109131)
View Research Applications

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

Neurobiology Research
Amyotrophic Lateral Sclerosis (ALS)
Cre-lox System
      loxP-flanked Sequences
Neurodegeneration

Research Tools
Cre-lox System
      loxP-flanked Sequences

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(SOD1*G37R)1Dwc
Allele Name transgene insertion 1, Don W Cleveland
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) LoxSOD1G37R;
Mutation Made ByDr. Don Cleveland,   Ludwig Institute for Cancer Res. (UCSD)
Strain of Origin(C57BL/6J x C3H/HeJ)F2
Expressed Gene SOD1, superoxide dismutase 1, soluble, human
Promoter SOD1, superoxide dismutase 1, soluble, human
General Note Mice develop fatal progressive motorneuron disease, including weight loss from denervation-induced muscle atrophy and paralysis. The highest expressing line reached end stage disease between 8.5 and 11 months. No human SOD1 was expressed in progeny from transgenic females that also expressed a germ line Cre transgene. The effects of mutant SOD1 within motorneurons was assessed by mating human mutant SOD1-expressing transgenic mice with mice expressing Cre under control of the Islet-1 promoter to remove expression from motorneurons specifically.
Molecular Note The SOD1*G37R transgene was designed with the entire human "floxed"-superoxide dismutase 1, soluble (SOD1) gene, modified to harbor the SOD1*G37R mutation, driven by its endogenous human promoter. The expressed protein is characterized as an enzymatically active, "gain of adverse function" mutation. When transgenic mice are bred to mice expressing tissue-specific Cre recombinase, the resulting offspring will have the floxed-SOD1*G37R sequence deleted in the cre-expressing tissues. [MGI Ref ID J:109131]
 

Genotyping

Genotyping Information

Genotyping Protocols

Sod TgN Copy Number, QPCR
Tg(SOD), Melt Curve Analysis


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Boillee S; Yamanaka K; Lobsiger CS; Copeland NG; Jenkins NA; Kassiotis G; Kollias G; Cleveland DW. 2006. Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia. Science 312(5778):1389-92. [PubMed: 16741123]  [MGI Ref ID J:109131]

Additional References

Tg(SOD1*G37R)1Dwc related

Chang Y; Kong Q; Shan X; Tian G; Ilieva H; Cleveland DW; Rothstein JD; Borchelt DR; Wong PC; Lin CL. 2008. Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. PLoS ONE 3(8):e2849. [PubMed: 18682740]  [MGI Ref ID J:140123]

Da Cruz S; Parone PA; Lopes VS; Lillo C; McAlonis-Downes M; Lee SK; Vetto AP; Petrosyan S; Marsala M; Murphy AN; Williams DS; Spiegelman BM; Cleveland DW. 2012. Elevated PGC-1alpha activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metab 15(5):778-86. [PubMed: 22560226]  [MGI Ref ID J:184777]

Furukawa Y; Kaneko K; Yamanaka K; O'Halloran TV; Nukina N. 2008. Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis. J Biol Chem 283(35):24167-76. [PubMed: 18552350]  [MGI Ref ID J:140373]

Kang SH; Li Y; Fukaya M; Lorenzini I; Cleveland DW; Ostrow LW; Rothstein JD; Bergles DE. 2013. Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. Nat Neurosci 16(5):571-9. [PubMed: 23542689]  [MGI Ref ID J:197615]

Lobsiger CS; Boillee S; McAlonis-Downes M; Khan AM; Feltri ML; Yamanaka K; Cleveland DW. 2009. Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Proc Natl Acad Sci U S A 106(11):4465-70. [PubMed: 19251638]  [MGI Ref ID J:146766]

Mishra A; Maheshwari M; Chhangani D; Fujimori-Tonou N; Endo F; Joshi AP; Jana NR; Yamanaka K. 2013. E6-AP association promotes SOD1 aggresomes degradation and suppresses toxicity. Neurobiol Aging 34(4):1310.e11-23. [PubMed: 23040663]  [MGI Ref ID J:203386]

Parone PA; Da Cruz S; Han JS; McAlonis-Downes M; Vetto AP; Lee SK; Tseng E; Cleveland DW. 2013. Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. J Neurosci 33(11):4657-71. [PubMed: 23486940]  [MGI Ref ID J:196358]

Yamanaka K; Chun SJ; Boillee S; Fujimori-Tonou N; Yamashita H; Gutmann DH; Takahashi R; Misawa H; Cleveland DW. 2008. Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci 11(3):251-3. [PubMed: 18246065]  [MGI Ref ID J:135268]

Zhong Z; Ilieva H; Hallagan L; Bell R; Singh I; Paquette N; Thiyagarajan M; Deane R; Fernandez JA; Lane S; Zlokovic AB; Liu T; Griffin JH; Chow N; Castellino FJ; Stojanovic K; Cleveland DW; Zlokovic BV. 2009. Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells. J Clin Invest 119(11):3437-49. [PubMed: 19841542]  [MGI Ref ID J:154597]

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, hemizygous mice may be bred to wildtype (noncarrier) mice from the colony or to C57BL/6NJ inbred mice (Stock No. 005304)

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2140.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We willfulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory 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. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2782.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We willfulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory 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. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J (approximate)
   005304 C57BL/6NJ (approximate)
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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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