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Strain Name:

B6.129S7-Sod2tm1Leb/J

Stock Number:

002973

Availability:

Repository- Live

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General Terms and Conditions

Genes & Alleles   Sod2;   Sod2tm1Leb;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Targeted Mutation
Mating SystemInbred x Heterozygote         (Female x Male)
Specieslaboratory mouse
Background Strain C57BL/6
Donor Strain 129S7 via AB2.1 ES cell line
Donating Investigator Russell Lebovitz,   SUMA Partners
GenerationN5+8 (04-DEC-07)

Appearance
black
Related Genotype: a/a

Strain Description
Mice homozygous for the Sod2tm1Leb targeted mutation die within 21 days after birth. They exhibit severe wasting and are clearly much smaller, weaker,and less coordinated than their wildtype and heterozygous littermates. Homoyzotes exhibit several novel pathogenic phenotypes including severe anemia, degeneration of neurons in the basal ganglia and brainstem, progressive motor disturbances, and myocardial injury.

Strain Development
The Sod2tm1Leb targeted mutation was made by Dr. Russell Lebovitz. A 1.5 Kb HindIII fragment containing exons 1 and 2 of the Sod2 gene, as well as approximately 500bp immediately 5' of exon 1, was replaced with a human hypoxanthine phosphoribosyltransferase minigene driven by the phosphoglycerate kinase promoter. The transcription and translation start sites, the mitochondrial targeting sequence, and one of the three histidines that bind directly to the manganese cofactor, have been eliminated. The 129S7/SvEvBrd-Hprtb-m2-derived AB 2.1 ES cell line was used. The Sod2tm1Leb targeted mutation has been backcrossed more than 5 times to C57BL/6 mice.

Mammalian Phenotype Terms assigned by genotype

Sod2tm1Leb/Sod2+

        B6.129S7-Sod2tm1Leb/J
  • hearing/vestibular/ear phenotype
  • *normal* hearing/vestibular/ear phenotype (MGI Ref ID J:119972)
    • aging heterozygotes exhibit no significant increase in the amount of age-related hearing loss relative to C57BL/6 control mice, with many animals over the age of 20 months being deaf or displaying ABR thresholds >80 dB SPL
    • contrary to expectation, ABR thresholds are only 12 dB higher in heterozygotes relative to C57BL/6 control mice (at various age groups including 6-9, 12, 15, 18-21 and 24 months and stimuli including click, 8, 16 and 32 kHz); no explanation for the relatively good hearing of three 24-month-old heterozygotes is provided

Sod2tm1Leb/Sod2tm1Leb

        B6.129S7-Sod2tm1Leb/J
  • lethality-postnatal
  • postnatal lethality (MGI Ref ID J:36148)
    • homozygotes survive for up to 3 weeks of age
    • mutants with the slowest growth rates (4 of 8) usually die during the first postnatal week
    • mutants with intermediate growth rates die within the second or third week
  • growth/size phenotype
  • decreased body size (MGI Ref ID J:36148)
    • at P10, homozygotes are significantly smaller than wild type littermates
    • however, no skeletal abnormalities are observed and failure to thrive is unrelated to nursing difficulties
    • decreased body length (MGI Ref ID J:36148)
    • decreased body weight (MGI Ref ID J:36148)
  • postnatal growth retardation (MGI Ref ID J:36148)
    • reduction of postnatal growth rate is variable among mice, first evident between P2 and P7, and progressive until death by P18
  • behavior/neurological phenotype
  • abnormal motor capabilities/coordination/movement (MGI Ref ID J:36148)
    • homozygotes display striking and progressive motor deficits
    • circling (MGI Ref ID J:36148)
    • weakness (MGI Ref ID J:36148)
      • homozygotes exhibit progressive limb weakness, as shown by impaired ability to hold onto a bar suspended above the cage for at least 5 sec without falling or scale a 60 degree incline
  • fatigue (MGI Ref ID J:36148)
    • homozygotes display earlier onset of fatigue, as shown by reduced endurance only after 2-3 cycles of immersion into a small room-temperature water bath (vs 10 cycles in wild type mice)
  • hematopoietic system phenotype
  • anemia (MGI Ref ID J:36148)
    • homozygotes are severely anemic at the time of death
  • decreased bone marrow cell number (MGI Ref ID J:36148)
    • homozygotes display a hypocellular bone marrow
  • impaired myelopoiesis (MGI Ref ID J:36148)
    • within the bone marrow, all hematopoietic lineages appear to be reduced
  • nervous system phenotype
  • neurodegeneration (MGI Ref ID J:36148)
    • homozygotes display degeneration of large CNS neurons, esp. in the basal ganglia and brainstem
    • neurodegeneration is associated with extensive mitochondrial damage, loss of polysomes, clearing of the cytoplasm, a relative absence of rough ER, focal dilation of smooth ER, and ruffling of nuclear membranes
    • in P10 basal ganglia, early cellular degeneration includes dispersion of cytoplasm, shedding of ribosomes, and balloning of mitochondria
    • in affected brain and brainstem regions, occasional swollen neurites are observed; however, most structures and mitochondria remain intact
  • cellular phenotype
  • abnormal mitochondrial physiology (MGI Ref ID J:36148)
    • at >P7, homozygotes show extensive mitochondrial injury in affected cardiac muscle cells and degenerating neurons
  • adipose tissue phenotype
  • decreased adipose tissue amount (MGI Ref ID J:36148)
    • homozygotes display a significant reduction in adipose tissue mass
  • muscle phenotype
  • decreased skeletal muscle mass (MGI Ref ID J:36148)
    • homozygotes display a significant reduction in skeletal muscle mass
  • dilated cardiomyopathy (MGI Ref ID J:36148)
    • only 10% of homozygotes display extreme baloon-like cardiac dilatation with thinning of the ventricular wall
    • affected myocytes exhibit widespread cellular injury and death associated with mitochondrial injury (swelling and fragmentation) and lipid peroxidation of mitochondrial membranes in severely affected resgions
  • immune system phenotype
  • impaired myelopoiesis (MGI Ref ID J:36148)
    • within the bone marrow, all hematopoietic lineages appear to be reduced
  • liver/biliary system phenotype
  • abnormal hepatocyte morphology (MGI Ref ID J:36148)
    • hepatic glycogen deposits appear coarsely granular and with a tendency towards centrilobular accumulation
    • in addition, an abundance of intracellular lipid vacuoles is observed
  • cardiovascular system phenotype
  • dilated cardiomyopathy (MGI Ref ID J:36148)
    • only 10% of homozygotes display extreme baloon-like cardiac dilatation with thinning of the ventricular wall
    • affected myocytes exhibit widespread cellular injury and death associated with mitochondrial injury (swelling and fragmentation) and lipid peroxidation of mitochondrial membranes in severely affected resgions
  • thin ventricular wall (MGI Ref ID J:36148)
    • homozygotes with extreme baloon-like cardiac dilatation (10%) display thinning of the ventricular wall
  • homeostasis/metabolism phenotype
  • abnormal lipid homeostasis (MGI Ref ID J:36148)
    • homozygotes display an abundance of intracellular lipid vacuoles in hepatocytes
    • homozygotes with extreme cardiac dilatation exhibit lipid peroxidation of mitochondrial membranes in affected myocytes
    • homozygotes without extreme cardiac dilatation show increased accumulation of neutral lipid in myocytes but no mitochondrial injury
  • hearing/vestibular/ear phenotype
  • circling (MGI Ref ID J:36148)
  • skin/coat/nails phenotype
  • pallor (MGI Ref ID J:36148)
    • homozygotes appear slightly pale

Gene & Allele Details

Allele Symbol Sod2tm1Leb
Allele Name targeted mutation 1, Russell M Lebovitz
Common Name(s) SOD2m1BCM; Sod2-; Sod2mlbcm;
Mutation Made By Russell Lebovitz,   SUMA Partners
Strain of Origin129S7/SvEvBrd-Hprt1
ES Cell Line NameAB2.1
ES Cell Line Strain129S7/SvEvBrd-Hprt1
Gene Symbol and Name Sod2, superoxide dismutase 2, mitochondrial
Chromosome 17
Gene Common Name(s) IPO-B; MGC:6144; MNSOD; Mn-SOD; Sod-2; manganese SOD; manganese superoxide dismutase;
Molecular Note A human HPRT minigene driven by the PGK promoter replaced exons 1 and 2, and sequences approximately 500 bp immediately 5' of exon 1. The replaced region encodes the transcription and translation start sites, the mitochondrial targeting sequence, and oneof three histidines that bind directly to the manganese cofactor. Northern blot analysis of brain did not detect mRNA in homozygous mutant mice. Enzyme activity assays of heart did not detect active protein in homozygous mutant mice. [MGI Ref ID J:36148]

Control Information

  Allele   Control
 Sod2tm1Leb  Wild-type from the colony
 Sod2tm1Leb  000664 C57BL/6J
 
  Considerations for Choosing Controls

Genotyping Protocols

Human HPRT

Colony Maintenance

Breeding & HusbandryThis strain is maintained by mating +/+ x heterozygous siblings, or heterozygous x +/+ siblings. Expected coat color from breeding:Black
Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying   Sod2tm1Leb allele
006883   B6.Cg-Ldlrtm1Her Sod2tm1Leb/J
View Strains carrying   Sod2tm1Leb     (1 strain)

Additional Web Information

Congenic Nomenclature

Animal Health Reports

Room Number           FGB29

Research Applications

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

Sod2tm1Leb related

Metabolism Research

Neurobiology Research
Metabolic Defects
Neurodegeneration

References

Selected Reference(s)

Lebovitz RM; Zhang H; Vogel H; Cartwright J Jr; Dionne L; Lu N; Huang S; Matzuk MM. 1996. Neurodegeneration, myocardial injury, and perinatal death in mitochondrial superoxide dismutase-deficient mice. Proc Natl Acad Sci U S A 93(18):9782-7. [PubMed: 8790408]  [MGI Ref ID J:36148]

Additional References

Price and Supply Information

Strain Name: B6.129S7-Sod2tm1Leb/J
Stock Number: 002973

Price Details

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Supply Details

Standard SupplyRepository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes Usually shipped between four and eight weeks of age.
This strain is included in the Induced Mutant Resource Colony collection.
Genomic DNA is available for this strain from the Mouse DNA Resource.
LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

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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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