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 old, hind limb tremors at 14 weeks old, 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 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 |
|
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 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
(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)
