| Primary Identifier | MGI:2671112 | Allele Type | Transgenic |
| Attribute String | Humanized sequence, Inserted expressed sequence | Gene |
Tg(SOD1*G93A)
|
| Strain of Origin | (C57BL/6 x SJL)F1 | Is Recombinase | false |
| Is Wild Type | false |
| description | 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. |
| molecularNote | 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. |
