Amyotrophic Lateral Sclerosis (ALS; Lou Gehrig's Disease)
Use this resource to identify and select the most appropriate mouse model of ALS for your research and to obtain a guide to help you efficiently work with these mice.
- Download the manual Working with ALS mice, guidelines for preclinical testing & colony management. (.pdf)
- See a complete list of JAX® Mice for ALS research
Comparison of featured ALS models
This table summarizes the differences between important mouse models for ALS to help you easily find the right strain for your research.
| Strain Name | Common Name | Molecular Mutation | Phenotype | Survival |
|---|---|---|---|---|
| B6SJL-Tg(SOD1*G93A)1Gur/J (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 of age, hind limb tremors at 14 weeks of age, become paralyzed in one or more limbs | 50% survival at 128.9+/-9.1 days |
| B6.Cg-Tg(SOD1*G93A)1Gur/J (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 human TAR DNA binding protein modified to harbor an N-terminal Flag tag immediately after the start methionine and the A315T amino acid substitution | Progressive and fatal neurodegenerative disease, frontotemporal lobar degeneration with ubiquitin aggregates | Average survival time of 97+/-11 days, females live longer than males |
| STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J (016143) |
A315T TDP-43 line 23 | Mouse prion promoter driving human TAR DNA binding protein with the A315T amino acid substitution | Late onset progressive motor phenotype, characterized by hind limb weakness, progressive decline in grip strength, and reduction in stride length | Average survival time of 75 days |
| B6SJL-Tg(SOD1)2Gur/J (002297) |
WT SOD1 | Normal human SOD1 | Normal | Normal |
Featured JAX® Mice Models of ALS
B6SJL-Tg(SOD1*G93A)1Gur/J
002726
- 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
004435
- 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
010700
- 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 time of approximately 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
STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J
016143
- Common name: A315T TDP-43 line 23 transgenic
- Genetic background: mixed; includes C57BL/6J (000664), SJL/J (000686) and CD1 outbred
- Expresses the full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA carrying the A315T amino acid substitution associated with familial ALS (Stallings et al. 2010)
- Develop a late onset, progressive motor phenotype, characterized by hind limb weakness, progressive decline in grip strength, and reduction in stride length
- Hemizygotes are fertile, with a mean survival of 75 days
B6SJL-Tg(SOD1)2Gur/J
002297
- 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)