These C57BL/6J-congenic SOD1-G93A transgenic mice carry a reduced copy number of the variant human superoxide dismutase 1 soluble gene transgene, Tg(SOD1*G93A)dl1Gur. These mice exhibit a delayed onset of phenotype when compared to the original high copy number C57BL/6J-congenic SOD1-G93A transgenic mice (B6.Cg-Tg(SOD1*G93A)1Gur/J; Stock No. 004435). These mice are useful for studies of neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS).
IMR Colony, The Jackson Laboratory
Genetic Background | Generation |
---|---|
000664 C57BL/6J |
|
Allele Type |
---|
Transgenic (Inserted expressed sequence, Humanized sequence) |
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 C57BL/6J-congenic SOD1-G93A transgenic mice (B6.Cg-Tg(SOD1*G93A)1Gur/J; Stock No. 004435) because of a reduction in transgenic copy number (see details below). 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 for five generations. 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.
In 2014, Genetic Quality Assurance/Control program at The Jackson Laboratory Repository assessed the survival of a cohort of hemizygous mice (16 females and 9 males; mixed siblings and non-siblings). This showed all tested animals survived beyond 200 days, ~50% survival at 275-300 days of age, and all tested animals die before one year of age. This confirms that reduced transgene copy number in these C57BL/6J-congenic SOD1-G93A transgenic mice (Stock No. 002299) results in a slower progressing model of ALS compared to the original high copy number C57BL/6J-congenic SOD1-G93A transgenic mice (Stock No. 004435; reported to have ~50% survival at 157 days of age).
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 five generations.
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 |
Chromosome | 12 |
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 |
When maintaining a live colony, hemizygous mice may be bred to wildtype siblings, or to C57BL/6J inbred mice (Stock No. 000664).
When using the G93A SOD1 mouse strain in a publication, please cite the originating article(s) and include JAX stock #002299 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service | Genotype | Price |
---|---|---|
Carrier or Non carrier for Tg(SOD1*G93A) |
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 | B6.Cg-Tg(SOD1*G93A)<dl>1Gur/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6.Cg-Tg(SOD1*G93A)<dl>1Gur/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6.Cg-Tg(SOD1*G93A)<dl>1Gur/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | B6.Cg-Tg(SOD1*G93A)<dl>1Gur/J Frozen Embryo | $3373.50 |
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.
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.
"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"). "PRODUCT(S)" means biological materials supplied by JACKSON, and their derivatives. "SERVICES" means projects conducted by JACKSON for other parties that may include but are not limited to the use of MICE or PRODUCTS. "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 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, PRODUCTS or SERVICES from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON’s prior written authorization.
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, PRODUCTS or SERVICES, JACKSON will, at its option, provide credit or replacement for the PRODUCT received or the SERVICES provided; JACKSON makes no other representations and this shall be the exclusive remedy of the purchaser. Please note specific policy for live mice.
Consistent with the requirement for a written understanding regarding animal care and use, the JACKSON Animal Care and Use Committee will review the animal care and use protocol(s) associated with any SERVICES to be performed at JACKSON, and JACKSON shall have ultimate responsibility and authority for the care of animals while on site or in JACKSON custody.
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, PRODUCTS or SERVICES 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.