Strain Name:

B6.Cg-Tg(SOD1*G93A)dl1Gur/J

Stock Number:

002299

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Use Restrictions Apply, see Terms of Use
These transgenic mice carry a reduced copy number of a transgene containing a variant of the human superoxide dismutase 1, soluble gene (SOD1), and exhibit a delayed onset of phenotype when compared to the higher copy number transgenic line, (B6SJL-Tg(SOD1*G93A)1Gur/J, Stock No. 002726). These mice are useful for studies of neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS).

Description

Strain Information

Former Names B6.Cg-Tg(SOD1-G93A)dl1Gur/J    (Changed: 11-JUL-07 )
Type Congenic; Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Mating SystemNoncarrier x Hemizygote         (Female x Male)   01-APR-13
Mating SystemHemizygote x Noncarrier         (Female x Male)   01-APR-13
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain Cg
GenerationN?+16p+N1pN2 (20-AUG-14)
Generation Definitions
 
Donating Investigator IMR Colony,   The Jackson Laboratory

Appearance
black
Related Genotype: a/a

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 strain (SOD1*G93A)1Gur, Stock No. 002726) because of a reduction in transgenic copy number. 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 5 times. 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.

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 5 generations.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

View Amyotrophic Lateral Sclerosis (ALS)     (30 strains)

Strains carrying   Tg(SOD1*G93A)dl1Gur allele
002300   B6SJL-Tg(SOD1*G93A)dl1Gur/J
View Strains carrying   Tg(SOD1*G93A)dl1Gur     (1 strain)

View Strains carrying other alleles of SOD1     (16 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*G93A)dl1Gur/0

        involves: C57BL/6 * CBA * SJL
  • mortality/aging
  • premature death
    • most symptomatic animals die at <300 days of age   (MGI Ref ID J:134095)
  • behavior/neurological phenotype
  • impaired coordination
    • inability to perform hanging wire test   (MGI Ref ID J:134095)
  • muscle phenotype
  • muscle weakness
    • first sign of disease is forelimb weakness   (MGI Ref ID J:134095)
    • progressive muscle weakness
      • 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   (MGI Ref ID J:134095)
      • animals show forelimb onset of disease   (MGI Ref ID J:134095)
  • cellular phenotype
  • abnormal mitochondrion morphology
    • vacuolated mitochondria are sometimes seen in spinal cord of end-stage animals   (MGI Ref ID J:134095)
  • nervous system phenotype
  • astrocytosis   (MGI Ref ID J:134095)

Tg(SOD1*G93A)dl1Gur/0

        involves: C57BL/6 * SJL
  • mortality/aging
  • premature death
  • 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   (MGI Ref ID J:106420)
  • 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   (MGI Ref ID J:106420)
  • behavior/neurological phenotype
  • limb grasping
    • develops at day 200   (MGI Ref ID J:106420)
  • paralysis   (MGI Ref ID J:155806)
    • hindlimb paralysis   (MGI Ref ID J:155806)
  • muscle phenotype
  • muscle weakness
    • develops at day 200   (MGI Ref ID J:106420)

Tg(SOD1*G93A)dl1Gur/0

        involves: C57BL/6 * C57BL/6J * SJL/J
  • nervous system phenotype
  • astrocytosis
    • increased numbers of reactive astrocytes in facial and hypoglossal nuclei at 8 months of age   (MGI Ref ID J:128738)
  • decreased motor neuron number
    • lower numbers of facial and hypoglossal motoneurons at 8 months of age   (MGI Ref ID J:128738)
  • microgliosis
    • increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age   (MGI Ref ID J:128738)
  • motor neuron degeneration
    • atrophic facial and hypoglossal motoneurons at 8 months of age   (MGI Ref ID J:128738)
  • immune system phenotype
  • microgliosis
    • increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age   (MGI Ref ID J:128738)
  • hematopoietic system phenotype
  • microgliosis
    • increased numbers of microglia in facial and hypoglossal nuclei at 8 months of age   (MGI Ref ID J:128738)
View Research Applications

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

Metabolism Research

Neurobiology Research
Amyotrophic Lateral Sclerosis (ALS)
Metabolic Defects
Neurodegeneration

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(SOD1*G93A)dl1Gur
Allele Name transgene insertion dl 1, Mark E Gurney
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) G1L; G93A SOD1; Tg(SOD1-G93A)dl1Gur; TgN(SOD1-G93A)1Gurdl; TgN(SOD1-G93A)1Gurdl; TgNSOD1-G93A; TgN[SOD1-G93A]dl1Gur; mSOD1-Tg;
Mutation Made ByDr. Mark Gurney,   Tetra Discovery Partners
Strain of Origin(C57BL/6 x SJL)F1
Expressed Gene SOD1, superoxide dismutase 1, soluble, human
Promoter SOD1, superoxide dismutase 1, soluble, human
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 backgroundinvolving 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. [MGI Ref ID J:106420] [MGI Ref ID J:32665]
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(SOD), Melt Curve Analysis


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

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):1772-5. [PubMed: 8209258]  [MGI Ref ID 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):1282-9. [PubMed: 11591849]  [MGI Ref ID 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):157-60. [PubMed: 11343826]  [MGI Ref ID 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):1363-70. [PubMed: 11298796]  [MGI Ref ID J:85032]

Tg(SOD1*G93A)dl1Gur related

Alexander GM; Erwin KL; Byers N; Deitch JS; Augelli BJ; Blankenhorn EP; Heiman-Patterson TD. 2004. Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS. Brain Res Mol Brain Res 130(1-2):7-15. [PubMed: 15519671]  [MGI Ref ID J:94198]

Alexianu ME; Kozovska M; Appel SH. 2001. Immune reactivity in a mouse model of familial ALS correlates with disease progression. Neurology 57(7):1282-9. [PubMed: 11591849]  [MGI Ref ID J:78936]

Ammassari-Teule M; Restivo L; Pietteur V; Passino E. 2001. Learning about the context in genetically-defined mice. Behav Brain Res 125(1-2):195-204. [PubMed: 11682111]  [MGI Ref ID J:92773]

Chang Q; Martin LJ. 2011. Glycine receptor channels in spinal motoneurons are abnormal in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci 31(8):2815-27. [PubMed: 21414903]  [MGI Ref ID J:180922]

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]

Copray JC; Jaarsma D; Kust BM; Bruggeman RW; Mantingh I; Brouwer N; Boddeke HW. 2003. Expression of the low affinity neurotrophin receptor p75 in spinal motoneurons in a transgenic mouse model for amyotrophic lateral sclerosis. Neuroscience 116(3):685-94. [PubMed: 12573711]  [MGI Ref ID J:132258]

Elliott JL. 2001. Cytokine upregulation in a murine model of familial amyotrophic lateral sclerosis. Brain Res Mol Brain Res 95(1-2):172-8. [PubMed: 11687290]  [MGI Ref ID J:72854]

Giess R; Holtmann B; Braga M; Grimm T; Muller-Myhsok B; Toyka KV; Sendtner M. 2002. Early onset of severe familial amyotrophic lateral sclerosis with a SOD-1 mutation: potential impact of CNTF as a candidate modifier gene. Am J Hum Genet 70(5):1277-86. [PubMed: 11951178]  [MGI Ref ID J:76257]

Holasek SS; Wengenack TM; Kandimalla KK; Montano C; Gregor DM; Curran GL; Poduslo JF. 2005. Activation of the stress-activated MAP kinase, p38, but not JNK in cortical motor neurons during early presymptomatic stages of amyotrophic lateral sclerosis in transgenic mice. Brain Res 1045(1-2):185-98. [PubMed: 15910777]  [MGI Ref ID J:131690]

Hoyaux D; Alao J; Fuchs J; Kiss R; Keller B; Heizmann CW; Pochet R; Frermann D. 2000. S100A6, a calcium- and zinc-binding protein, is overexpressed in SOD1 mutant mice, a model for amyotrophic lateral sclerosis. Biochim Biophys Acta 1498(2-3):264-72. [PubMed: 11108968]  [MGI Ref ID J:66524]

Inoue H; Tsukita K; Iwasato T; Suzuki Y; Tomioka M; Tateno M; Nagao M; Kawata A; Saido TC; Miura M; Misawa H; Itohara S; Takahashi R. 2003. The crucial role of caspase-9 in the disease progression of a transgenic ALS mouse model. EMBO J 22(24):6665-74. [PubMed: 14657037]  [MGI Ref ID J:132573]

Jaarsma D; Teuling E; Haasdijk ED; De Zeeuw CI; Hoogenraad CC. 2008. Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice. J Neurosci 28(9):2075-88. [PubMed: 18305242]  [MGI Ref ID J:134095]

Joosten EA; Van Westerlaak MG; Biesheuvel C; Woodhams PL; Brook GA; Veldman H; Bar PR. 2001. Cellular changes in motoneurons in a transgenic mouse model for amyotrophic lateral sclerosis as revealed by monoclonal antibody Py. Brain Res Dev Brain Res 131(1-2):153-9. [PubMed: 11718846]  [MGI Ref ID J:72961]

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):157-60. [PubMed: 11343826]  [MGI Ref ID J:85031]

Kabuta T; Kinugawa A; Tsuchiya Y; Kabuta C; Setsuie R; Tateno M; Araki T; Wada K. 2009. Familial amyotrophic lateral sclerosis-linked mutant SOD1 aberrantly interacts with tubulin. Biochem Biophys Res Commun 387(1):121-6. [PubMed: 19576169]  [MGI Ref ID J:151630]

Kadoyama K; Funakoshi H; Ohya W; Nakamura T. 2007. Hepatocyte growth factor (HGF) attenuates gliosis and motoneuronal degeneration in the brainstem motor nuclei of a transgenic mouse model of ALS. Neurosci Res 59(4):446-56. [PubMed: 17935811]  [MGI Ref ID J:128738]

Kong J; Xu Z. 2000. Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis. Neurosci Lett 281(1):72-4. [PubMed: 10686419]  [MGI Ref ID J:60455]

Maatkamp A; Vlug A; Haasdijk E; Troost D; French PJ; Jaarsma D. 2004. Decrease of Hsp25 protein expression precedes degeneration of motoneurons in ALS-SOD1 mice. Eur J Neurosci 20(1):14-28. [PubMed: 15245475]  [MGI Ref ID J:100257]

Mandillo S; Heise I; Garbugino L; Tocchini-Valentini GP; Giuliani A; Wells S; Nolan PM. 2014. Early motor deficits in mouse disease models are reliably uncovered using an automated home-cage wheel-running system: a cross-laboratory validation. Dis Model Mech 7(3):397-407. [PubMed: 24423792]  [MGI Ref ID J:208817]

Martin LJ; Gertz B; Pan Y; Price AC; Molkentin JD; Chang Q. 2009. The mitochondrial permeability transition pore in motor neurons: Involvement in the pathobiology of ALS mice. Exp Neurol 218(2):333-46. [PubMed: 19272377]  [MGI Ref ID J:151275]

Morimoto N; Nagai M; Ohta Y; Miyazaki K; Kurata T; Morimoto M; Murakami T; Takehisa Y; Ikeda Y; Kamiya T; Abe K. 2007. Increased autophagy in transgenic mice with a G93A mutant SOD1 gene. Brain Res 1167:112-7. [PubMed: 17689501]  [MGI Ref ID J:124927]

Nadeau JH. 2003. Modifier genes and protective alleles in humans and mice. Curr Opin Genet Dev 13(3):290-5. [PubMed: 12787792]  [MGI Ref ID J:88012]

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):1363-70. [PubMed: 11298796]  [MGI Ref ID J:85032]

Okuno T; Nakatsuji Y; Kumanogoh A; Koguchi K; Moriya M; Fujimura H; Kikutani H; Sakoda S. 2004. Induction of cyclooxygenase-2 in reactive glial cells by the CD40 pathway: relevance to amyotrophic lateral sclerosis. J Neurochem 91(2):404-12. [PubMed: 15447673]  [MGI Ref ID J:93256]

Proescher JB; Son M; Elliott JL; Culotta VC. 2008. Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS. Hum Mol Genet 17(12):1728-37. [PubMed: 18337307]  [MGI Ref ID J:135987]

Prudlo J; Koenig J; Graser J; Burckhardt E; Mestres P; Menger M; Roemer K. 2000. Motor neuron cell death in a mouse model of FALS is not mediated by the p53 cell survival regulator Brain Res 879(1-2):183-7. [PubMed: 11011020]  [MGI Ref ID J:65103]

Puttaparthi K; Elliott JL. 2005. Non-neuronal induction of immunoproteasome subunits in an ALS model: possible mediation by cytokines. Exp Neurol 196(2):441-51. [PubMed: 16242125]  [MGI Ref ID J:104528]

Puttaparthi K; Gitomer WL; Krishnan U; Son M; Rajendran B; Elliott JL. 2002. Disease progression in a transgenic model of familial amyotrophic lateral sclerosis is dependent on both neuronal and non-neuronal zinc binding proteins. J Neurosci 22(20):8790-6. [PubMed: 12388585]  [MGI Ref ID J:79760]

Puttaparthi K; Van Kaer L; Elliott JL. 2007. Assessing the role of immuno-proteasomes in a mouse model of familial ALS. Exp Neurol 206(1):53-8. [PubMed: 17482163]  [MGI Ref ID J:141580]

Puttaparthi K; Wojcik C; Rajendran B; DeMartino GN; Elliott JL. 2003. Aggregate formation in the spinal cord of mutant SOD1 transgenic mice is reversible and mediated by proteasomes. J Neurochem 87(4):851-60. [PubMed: 14622116]  [MGI Ref ID J:86631]

Reinholz MM; Merkle CM; Poduslo JF. 1999. Therapeutic benefits of putrescine-modified catalase in a transgenic mouse model of familial amyotrophic lateral sclerosis. Exp Neurol 159(1):204-16. [PubMed: 10486188]  [MGI Ref ID J:57736]

Sasaki S; Warita H; Komori T; Murakami T; Abe K; Iwata M. 2006. Parvalbumin and calbindin D-28k immunoreactivity in transgenic mice with a G93A mutant SOD1 gene. Brain Res 1083(1):196-203. [PubMed: 16546142]  [MGI Ref ID J:108189]

Shefner JM; Reaume AG; Flood DG; Scott RW; Kowall NW; Ferrante RJ; Siwek DF; Upton-Rice M; Brown RH Jr. 1999. Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy. Neurology 53(6):1239-46. [PubMed: 10522879]  [MGI Ref ID J:59714]

Son M; Puttaparthi K; Kawamata H; Rajendran B; Boyer PJ; Manfredi G; Elliott JL. 2007. Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology. Proc Natl Acad Sci U S A 104(14):6072-7. [PubMed: 17389365]  [MGI Ref ID J:120361]

Stallings NR; Puttaparthi K; Dowling KJ; Luther CM; Burns DK; Davis K; Elliott JL. 2013. TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis. PLoS One 8(8):e71793. [PubMed: 23967244]  [MGI Ref ID J:206353]

Sun W; Funakoshi H; Nakamura T. 2002. Overexpression of HGF retards disease progression and prolongs life span in a transgenic mouse model of ALS. J Neurosci 22(15):6537-48. [PubMed: 12151533]  [MGI Ref ID J:78090]

Tamai S; Imaizumi K; Kurabayashi N; Nguyen MD; Abe T; Inoue M; Fukada Y; Sanada K. 2014. Neuroprotective role of the basic leucine zipper transcription factor NFIL3 in models of amyotrophic lateral sclerosis. J Biol Chem 289(3):1629-38. [PubMed: 24280221]  [MGI Ref ID J:207176]

Tateno M; Kato S; Sakurai T; Nukina N; Takahashi R; Araki T. 2009. Mutant SOD1 impairs axonal transport of choline acetyltransferase and acetylcholine release by sequestering KAP3. Hum Mol Genet 18(5):942-55. [PubMed: 19088126]  [MGI Ref ID J:145001]

Tateno M; Sadakata H; Tanaka M; Itohara S; Shin RM; Miura M; Masuda M; Aosaki T; Urushitani M; Misawa H; Takahashi R. 2004. Calcium-permeable AMPA receptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model. Hum Mol Genet 13(19):2183-96. [PubMed: 15294873]  [MGI Ref ID J:93621]

Tokuda E; Ono S; Ishige K; Watanabe S; Okawa E; Ito Y; Suzuki T. 2008. Ammonium tetrathiomolybdate delays onset, prolongs survival, and slows progression of disease in a mouse model for amyotrophic lateral sclerosis. Exp Neurol 213(1):122-8. [PubMed: 18617166]  [MGI Ref ID J:138864]

Tokuda E; Ono S; Ishige K; Watanabe S; Okawa E; Ito Y; Suzuki T. 2007. Dysequilibrium between caspases and their inhibitors in a mouse model for amyotrophic lateral sclerosis. Brain Res 1148:234-42. [PubMed: 17397813]  [MGI Ref ID J:121618]

Tucci V; Achilli F; Blanco G; Lad HV; Wells S; Godinho S; Nolan PM. 2007. Reaching and grasping phenotypes in the mouse (Mus musculus): a characterization of inbred strains and mutant lines. Neuroscience 147(3):573-82. [PubMed: 17574766]  [MGI Ref ID J:124220]

Tummala H; Jung C; Tiwari A; Higgins CM; Hayward LJ; Xu Z. 2005. Inhibition of chaperone activity is a shared property of several Cu,Zn-superoxide dismutase mutants that cause amyotrophic lateral sclerosis. J Biol Chem 280(18):17725-31. [PubMed: 15753080]  [MGI Ref ID J:99092]

Urushitani M; Sik A; Sakurai T; Nukina N; Takahashi R; Julien JP. 2006. Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci 9(1):108-18. [PubMed: 16369483]  [MGI Ref ID J:105360]

Veldink JH; Bar PR; Joosten EA; Otten M; Wokke JH; van den Berg LH. 2003. Sexual differences in onset of disease and response to exercise in a transgenic model of ALS. Neuromuscul Disord 13(9):737-43. [PubMed: 14561497]  [MGI Ref ID J:128781]

Warita H; Hayashi T; Murakami T; Manabe Y; Abe K. 2001. Oxidative damage to mitochondrial DNA in spinal motoneurons of transgenic ALS mice. Brain Res Mol Brain Res 89(1-2):147-52. [PubMed: 11311985]  [MGI Ref ID J:134551]

Warita H; Itoyama Y; Abe K. 1999. Selective impairment of fast anterograde axonal transport in the peripheral nerves of asymptomatic transgenic mice with a G93A mutant SOD1 gene. Brain Res 819(1-2):120-31. [PubMed: 10082867]  [MGI Ref ID J:53731]

Wengenack TM; Holasek SS; Montano CM; Gregor D; Curran GL; Poduslo JF. 2004. Activation of programmed cell death markers in ventral horn motor neurons during early presymptomatic stages of amyotrophic lateral sclerosis in a transgenic mouse model. Brain Res 1027(1-2):73-86. [PubMed: 15494159]  [MGI Ref ID J:93525]

Williams AH; Valdez G; Moresi V; Qi X; McAnally J; Elliott JL; Bassel-Duby R; Sanes JR; Olson EN. 2009. MicroRNA-206 delays ALS progression and promotes regeneration of neuromuscular synapses in mice. Science 326(5959):1549-54. [PubMed: 20007902]  [MGI Ref ID J:155806]

Wu DC; Re DB; Nagai M; Ischiropoulos H; Przedborski S. 2006. The inflammatory NADPH oxidase enzyme modulates motor neuron degeneration in amyotrophic lateral sclerosis mice. Proc Natl Acad Sci U S A 103(32):12132-7. [PubMed: 16877542]  [MGI Ref ID J:111782]

Zhang B; Tu P; Abtahian F; Trojanowski JQ; Lee VM. 1997. Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation. J Cell Biol 139(5):1307-15. [PubMed: 9382875]  [MGI Ref ID J:106420]

von Lewinski F; Fuchs J; Vanselow BK; Keller BU. 2008. Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice. Neurosci Lett 445(3):224-8. [PubMed: 18782598]  [MGI Ref ID J:142609]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX12

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, hemizygous mice may be bred to wildtype siblings, or to C57BL/6J inbred mice (Stock No. 000664).
Mating SystemNoncarrier x Hemizygote         (Female x Male)   01-APR-13
Hemizygote x Noncarrier         (Female x Male)   01-APR-13

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 $232.00Female or MaleHemizygous for Tg(SOD1*G93A)dl1Gur  
Price per Pair (US dollars $)Pair Genotype
$304.00Hemizygous for Tg(SOD1*G93A)dl1Gur x Noncarrier  
$304.00Noncarrier x Hemizygous for Tg(SOD1*G93A)dl1Gur  

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 $301.60Female or MaleHemizygous for Tg(SOD1*G93A)dl1Gur  
Price per Pair (US dollars $)Pair Genotype
$395.20Hemizygous for Tg(SOD1*G93A)dl1Gur x Noncarrier  
$395.20Noncarrier x Hemizygous for Tg(SOD1*G93A)dl1Gur  

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.

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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.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

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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Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
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Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license.

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General inquiries regarding Terms of Use

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phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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