These rederived G93A-SOD1 transgenic mice carry a lower copy number than the original transgenic strain and may be useful in studying neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease).
Dr. Mark E. Gurney, Tetra Discovery Partners
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 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.
This transgenic was made in the laboratory of Dr. Mark E. Gurney at Northwestern University. The transgene is the mutant human SOD1 containing the Gly93 -->Ala (G93A) substitution.
|Expressed Gene||SOD1, superoxide dismutase 1, human|
|Site of Expression|
|Allele Name||transgene insertion dl 1, Mark E Gurney|
|Allele Type||Transgenic (Inserted expressed sequence, Humanized sequence)|
|Allele Synonym(s)||transgene insertion dl 1, Mark E Gurney; Tg(SOD1*G93A)dl1Gur|
|Gene Symbol and Name||Tg(SOD1*G93A)dl1Gur, transgene insertion dl 1, Mark E Gurney|
|Gene Synonym(s)||G93A SOD1; mSOD1-Tg; Tg(SOD1-G93A)dl1Gur; TgN(SOD1-G93A)1Gurdl; TgNSOD1-G93A; TgN(SOD1-G93A)1Gurdl; TgN[SOD1-G93A]dl1Gur; G1L|
|Promoter||SOD1, superoxide dismutase 1, human|
|Expressed Gene||SOD1, superoxide dismutase 1, human|
|Strain of Origin||(C57BL/6 x SJL)F1|
|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 background involving 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.|
|Mutations Made By|| |
Dr. Mark Gurney, Tetra Discovery Partners
This strain is maintained by mating hemizygous carriers (preferably males) to B6SJLF1 hybrids. We have established an 8 month lifetime maximum for this strain that is compatible with The Jackson Laboratory policy for humane husbandry (i.e. paralysis has affected the mouse to the extent that it can not eat, drink, or move freely). Expected coat color from breeding;Black, White Bellied Agouti, Albino, Light Gray and Tan w/ pink eyes
When using the G93A SOD1 mouse strain in a publication, please cite the originating article(s) and include JAX stock #002300 in your Materials and Methods section.
|Hemizygous or Non carrier for Tg(SOD1*G93A)<dl>1Gur|
We will fulfill your order by providing at least two carriers for each strain ordered. The total number, sex, and genotypes provided will vary, although typically 8 or more animals are provided. Please check genotypes which will be recovered. While the genotypes of all animals produced will be communicated to you prior to scheduling shipment, the genotypes of animals provided may not reflect the mating scheme and genotypes described in the strain description. Animals are typically ready to ship in 11-14 weeks. If a second recovery is required to produce the minimum number of animals, then delivery time would increase to approximately 25 weeks. If we fail to produce animals of the correct genotype, you will not be charged. We 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.
|Frozen Mouse Embryo||$2,595.00 per straw or vial|
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