Amyotrophic Lateral Sclerosis Mouse Model Resource
Use this website to identify and select the most appropriate mouse model of ALS for your research and to find helpful resources..
- See a complete list of JAX® Mice for ALS research
See the Guide to Working with ALS Mice - Learn about ALS Efficacy Studies
Comparison table of featured ALS models
This table summarizes the differences among important mouse models for ALS to help you easily find the right strain for your research.
|
Strain Name |
Common Name |
Molecular Mutation |
Phenotype |
Survival |
|---|---|---|---|---|
|
002726 |
SOD1-G93A | Human SOD1 with glycine to alanine transition at position 93 | Decreased grip strength, impaired coordination, motor neuron degeneration, severe muscle weakness beyond 3 months old, hind limb tremors at 14 weeks old, become paralyzed in one or more limbs | 50% survival at 128.9+/-9.1 days |
|
004435
|
B6 SOD1-G93A | Human SOD1 with glycine to alanine transition at position 93 | Decreased grip strength, impaired coordination, motor neuron degeneration, decreased muscle size, hind limb tremors at 14 weeks of age, become paralyzed in one or more limbs | 50% survival at 157.1+/-9.3 days |
|
B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J 010700
|
Prp-TDP43-A315T | Mouse prion promoter driving a modified human TAR DNA binding protein with an A315T amino acid substitution | Progressive and fatal neurodegenerative disease, frontotemporal lobar degeneration with ubiquitin aggregates | Average survival: 97+/-11 days, females live longer than males |
|
002297 |
WT SOD1 | Normal human SOD1 | Normal | Normal |
Featured JAX® Mice Models of ALS
B6SJL-Tg(SOD1*G93A)1Gur/J
- Common name: SOD1-G93A transgenic
- Genetic background: mixed; containing C57BL/6J (000664) and SJL/J (000686)
- Expresses mutant human SOD1 with a glycine to alanine transition at position 93 (G93A) (Gurney et al. 1995)
- Become paralyzed in one or more limbs with paralysis due to loss of motor neurons from the spinal cord
- Abbreviated life span: 50% survival at 128.9+/-9.1 days
B6.Cg-Tg(SOD1*G93A)1Gur/J
- Common name: B6 SOD1-G93A
- Genetic background: C57BL/6J (000664); fully congenic
- Expresses mutant human SOD1 with a glycine to alanine transition at position 93 (G93A) (Gurney et al. 1995)
- Become paralyzed in one or more limbs with paralysis due to loss of motor neurons from the spinal cord
- Abbreviated life span: 50% survival at 157.1+/-9.3 (delayed compared to 002726)
B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J
- Common name: Prp-TDP43-A315T transgenic
- Genetic Background: C57BL/6J (000664); fully congenic
- Expresses a modified full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence carrying the A315T amino acid substitution associated with familial ALS and an N-terminal Flag tag immediately after the start methionine (Wegorzewska et al. 2009)
- Hemizygous males have an average survival of 3.5 months (97 +/- 11 days); this is earlier lethality than hemizygous males on a mixed C57BL/6;CBA genetic background
- Hemizygous females live significantly longer than hemizygous males
- View survival curve comparing hemizygous males and females
B6SJL-Tg(SOD1)2Gur/J
- Common name: WT SOD1 transgenic
- Genetic background: mixed, containing C57BL/6J (000664) and SJL/J (000686)
- Expresses normal human SOD1 (Gurney et al. 1995)
- SOD1 protein level is the same as in the transgenic strain carrying the SOD1*G93A transgene (002726)
- Control for B6SJL-Tg(SOD1*G93A)1Gur/J (002726) and B6SJL-Tg(SOD1*G93A)dl1Gur/J (002300)