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.

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

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

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)

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

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)