Strain Name:

B6.Cg-Tg(SOD1*G37R)42Dpr/J

Stock Number:

008342

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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.

Description

Strain Information

Type Congenic; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Mating SystemNoncarrier x Hemizygote         (Female x Male)   20-AUG-08
Specieslaboratory mouse
GenerationN6+N14 (11-DEC-13)
Generation Definitions
 
Donating InvestigatorDr. Don Cleveland,   Ludwig Institute for Cancer Res. (UCSD)

Description
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.

Development
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.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J
 
  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.

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
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
Amyotrophic Lateral Sclerosis 1; ALS1
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Tg(SOD1*G37R)42Dpr/0

        involves: C3H/HeJ * C57BL/6J
  • nervous system phenotype
  • abnormal spinal cord morphology
    • mice exhibit vacuolization in the neutropil of substantia gelatinosa and scattered in some of the fiber tracts of the ventral and lateral white matter of the spinal cord   (MGI Ref ID J:69178)
    • decreased motor neuron number
      • at late stages   (MGI Ref ID J:69178)
    • motor neuron degeneration
      • motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem   (MGI Ref ID J:69178)
      • degeneration is associated with vacuole formation in both dendrites and axons of motor neurons   (MGI Ref ID J:69178)
      • mice exhibit axonal degradation in dorsal roots of motor neurons   (MGI Ref ID J:69178)
  • brain vacuoles
    • in the choroid plexus   (MGI Ref ID J:69178)
  • behavior/neurological phenotype
  • hindlimb paralysis
    • eventually mice develop hindlimb paralysis   (MGI Ref ID J:69178)
  • hypoactivity
    • at 3.5 to 4 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice   (MGI Ref ID J:69178)
  • tremors
    • at 3.5 to 4 months of age, mice exhibit axial tremors   (MGI Ref ID J:69178)
  • weakness
    • at 3.5 to 4 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs   (MGI Ref ID J:69178)
  • muscle phenotype
  • abnormal muscle electrophysiology
    • mice exhibit spontaneous, positive sharp waves associated with denervation atrophy   (MGI Ref ID J:69178)
  • muscular atrophy
    • at 3.5 to 4 months of age, mice exhibit muscle wasting particularly along the flanks   (MGI Ref ID J:69178)
  • cellular phenotype
  • abnormal mitochondrion morphology
    • motor neurons exhibit mitochondrial degradation   (MGI Ref ID J:69178)
  • growth/size/body phenotype
  • weight loss
    • progressive   (MGI Ref ID J:69178)
  • renal/urinary system phenotype
  • abnormal proximal convoluted tubule morphology
    • mice exhibit vacuolization of epithelial cells in the proximal tubules of the kidney   (MGI Ref ID J:69178)
  • integument phenotype
  • rough coat
    • at 3.5 to 4 months of age   (MGI Ref ID J:69178)
View Research Applications

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

Neurobiology Research
Amyotrophic Lateral Sclerosis (ALS)
Neurodegeneration

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(SOD1*G37R)42Dpr
Allele Name transgene insertion 42, Donald L Price
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) G37R SOD1 line 42; G37R(42); SOD1 G37R line 42; Tg(SOD1-G37R)42Dpr;
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
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). [MGI Ref ID J:69178]
 

Genotyping

Genotyping Information

Genotyping Protocols

Sod TgN Copy Number, QPCR
Tg(SOD), Melt Curve Analysis
Tg(SOD1), Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Wong PC; Pardo CA; Borchelt DR; Lee MK; Copeland NG; Jenkins NA; Sisodia SS; Cleveland DW; Price DL. 1995. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14(6):1105-16. [PubMed: 7605627]  [MGI Ref ID J:69178]

Additional References

Tg(SOD1*G37R)42Dpr related

Beaulieu JM; Nguyen MD; Julien JP. 1999. Late onset death of motor neurons in mice overexpressing wild-type peripherin J Cell Biol 147(3):531-44. [PubMed: 15132161]  [MGI Ref ID J:58388]

Borchelt DR; Lee MK; Slunt HS; Guarnieri M; Xu ZS; Wong PC; Brown RH Jr; Price DL; Sisodia SS; Cleveland DW. 1994. Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity. Proc Natl Acad Sci U S A 91(17):8292-6. [PubMed: 8058797]  [MGI Ref ID J:133801]

Bruijn LI; Beal MF; Becher MW; Schulz JB; Wong PC; Price DL; Cleveland DW. 1997. Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxide dismutase 1 mutant. Proc Natl Acad Sci U S A 94(14):7606-11. [PubMed: 9207139]  [MGI Ref ID J:133804]

Chen X; Zhang X; Li C; Guan T; Shang H; Cui L; Li XM; Kong J. 2013. S-nitrosylated protein disulfide isomerase contributes to mutant SOD1 aggregates in amyotrophic lateral sclerosis. J Neurochem 124(1):45-58. [PubMed: 23043510]  [MGI Ref ID J:191002]

Chiu IM; Phatnani H; Kuligowski M; Tapia JC; Carrasco MA; Zhang M; Maniatis T; Carroll MC. 2009. Activation of innate and humoral immunity in the peripheral nervous system of ALS transgenic mice. Proc Natl Acad Sci U S A 106(49):20960-5. [PubMed: 19933335]  [MGI Ref ID J:155542]

Eyer J; Cleveland DW; Wong PC; Peterson AC. 1998. Pathogenesis of two axonopathies does not require axonal neurofilaments. Nature 391(6667):584-7. [PubMed: 9468135]  [MGI Ref ID J:91502]

Ilieva HS; Yamanaka K; Malkmus S; Kakinohana O; Yaksh T; Marsala M; Cleveland DW. 2008. Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci U S A 105(34):12599-604. [PubMed: 18719118]  [MGI Ref ID J:138825]

Lobsiger CS; Boillee S; Cleveland DW. 2007. Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A 104(18):7319-26. [PubMed: 17463094]  [MGI Ref ID J:133802]

Lobsiger CS; Boillee S; Pozniak C; Khan AM; McAlonis-Downes M; Lewcock JW; Cleveland DW. 2013. C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice. Proc Natl Acad Sci U S A 110(46):E4385-92. [PubMed: 24170856]  [MGI Ref ID J:202888]

Marinkovic P; Reuter MS; Brill MS; Godinho L; Kerschensteiner M; Misgeld T. 2012. Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 109(11):4296-301. [PubMed: 22371592]  [MGI Ref ID J:182236]

Nguyen MD; Lariviere RC; Julien JP. 2001. Deregulation of Cdk5 in a mouse model of ALS: toxicity alleviated by perikaryal neurofilament inclusions. Neuron 30(1):135-47. [PubMed: 11343650]  [MGI Ref ID J:69136]

Woodruff TM; Lee JD; Noakes PG. 2014. Role for terminal complement activation in amyotrophic lateral sclerosis disease progression. Proc Natl Acad Sci U S A 111(1):E3-4. [PubMed: 24381160]  [MGI Ref ID J:206365]

Yamanaka K; Boillee S; Roberts EA; Garcia ML; McAlonis-Downes M; Mikse OR; Cleveland DW; Goldstein LS. 2008. Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice. Proc Natl Acad Sci U S A 105(21):7594-9. [PubMed: 18492803]  [MGI Ref ID J:136374]

Zhong Z; Deane R; Ali Z; Parisi M; Shapovalov Y; O'Banion MK; Stojanovic K; Sagare A; Boillee S; Cleveland DW; Zlokovic BV. 2008. ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci 11(4):420-2. [PubMed: 18344992]  [MGI Ref ID J:136100]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX10

Colony Maintenance

Breeding & HusbandryMutant 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.
Mating SystemNoncarrier x Hemizygote         (Female x Male)   20-AUG-08
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $239.00Female or MaleHemizygous for Tg(SOD1*G37R)42Dpr  
Price per Pair (US dollars $)Pair Genotype
$311.00Noncarrier x Hemizygous for Tg(SOD1*G37R)42Dpr  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $310.70Female or MaleHemizygous for Tg(SOD1*G37R)42Dpr  
Price per Pair (US dollars $)Pair Genotype
$404.30Noncarrier x Hemizygous for Tg(SOD1*G37R)42Dpr  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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