JAX Mouse Strain Datasheet - 008342

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

Stock No:: 008342 |; G37R(42) SOD1

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Overview

Also Known As: G37R(42) SOD1

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.

Donating Investigator

Dr. Don Cleveland, Ludwig Institute for Cancer Research (UCSD)

Genetic overview

Genetic Background	Generation
	N6+N29F1 (20-DEC-16)

Tg(SOD1*G37R)42Dpr

Allele Type
Transgenic (Humanized sequence, Inserted expressed sequence)

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Research Applications

Neurobiology Research

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Base Price

Starting at:

$263.00 Domestic price for female

$337.00 Domestic price for breeder pair

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Details

Detailed 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.

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

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: J:69178

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Genetics

Tg(SOD1*G37R)42Dpr

Allele Symbol: Tg(SOD1*G37R)42Dpr

Allele Name	transgene insertion 42, Donald L Price
Allele Type	Transgenic (Humanized sequence, Inserted expressed 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)	G37R SOD1 line 42; G37R(42); SOD1 G37R line 42; Tg(SOD1-G37R)42Dpr; Tg(SOD1-G37R)42Dpr
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)

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Neurobiology Research
- Amyotrophic Lateral Sclerosis (ALS)
- Neurodegeneration

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(SOD1G37R)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

brain vacuoles
- in the choroid plexus

decreased motor neuron number
- at late stages

motor neuron degeneration
- degeneration is associated with vacuole formation in both dendrites and axons of motor neurons
- mice exhibit axonal degradation in dorsal roots of motor neurons
- motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem

behavior/neurological phenotype

hindlimb paralysis
- eventually mice develop hindlimb paralysis

hypoactivity
- at 3.5 to 4 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice

tremors
- at 3.5 to 4 months of age, mice exhibit axial tremors

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

muscle phenotype

abnormal muscle electrophysiology
- mice exhibit spontaneous, positive sharp waves associated with denervation atrophy

muscular atrophy
- at 3.5 to 4 months of age, mice exhibit muscle wasting particularly along the flanks

cellular phenotype

abnormal mitochondrion morphology
- motor neurons exhibit mitochondrial degradation

growth/size/body region phenotype

weight loss
- progressive

renal/urinary system phenotype

abnormal proximal convoluted tubule morphology
- mice exhibit vacuolization of epithelial cells in the proximal tubules of the kidney

integument phenotype

rough coat
- at 3.5 to 4 months of age

References

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: J:69178

Additional - 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: 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: 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: 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: 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: 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: 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: 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: 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: 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: 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: 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: 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: 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: J:136100

Pricing & Availability

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Live Mouse

Age

Genotype

Price

weeks

Breeder Pair

Sex

Genotype

Price

Female
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Cryorecovery - Pricing

Service	Genotype	Price
	Hemizygous or Non carrierfor Tg(SOD1*G37R)42Dpr

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 will fulfill 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.

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.

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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: G37R(42) SOD1

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Tg(SOD1*G37R)42Dpr

Allele Symbol: Tg(SOD1*G37R)42Dpr

Disease Terms

Research Areas By Genotype

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

Mammalian Phenotype Terms by Genotype

Genotype: Tg(SOD1*G37R)42Dpr/0involves: C3H/HeJ * C57BL/6J

nervous system phenotype

behavior/neurological phenotype

muscle phenotype

cellular phenotype

growth/size/body region phenotype

renal/urinary system phenotype

integument phenotype

References

Additional - Tg(SOD1*G37R)42Dpr related

Genotyping Protocols

Dietary Information

Breeding Considerations

Mating System

Citation

Animal Health Reports

Live Mouse

Breeder Pair

Cryorecovery - Pricing

Progeny testing is not required

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

Genotype: Tg(SOD1G37R)42Dpr/0
involves: C3H/HeJ C57BL/6J