B6SJL-Tg(SOD1*G93A)^dl1Gur/J

Stock number: 002300
Product name: G93A SOD1
Research areas: Metabolism Research, Mouse/Human Gene Homologs, Neurobiology Research

Description

These rederived G93A-SOD1 transgenic mice carry a lower copy number than the original transgenic strain and may be useful in studying neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease).

Detailed description

Mice carrying the transgene exhibit paralysis resulting in a reduced life expectancy. They serve as a model for human amyotrophic lateral sclerosis (ALS). The transgene carries a variant of the human Superoxide dismutase-1 gene (glycine to alanine at position 93). Paralysis is due to loss of motor neurons from the spinal cord. The onset of the ALS phenotype is delayed compared to the original high copy number strain (SOD1*G93A)1Gur, Stock No. 002726) because of a reduction in transgenic copy number. This reduction most likely occurred during the importation and establishment of the original high copy number mutant transgenic strain. Mice become paralyzed in one or more limbs beginning around six to seven months of age. Life expectancy is normally four to six weeks beyond onset of symptoms.

Development

This transgenic was made in the laboratory of Dr. Mark E. Gurney at Northwestern University. The transgene is the mutant human SOD1 containing the Gly⁹³ -->Ala (G93A) substitution.

Allele

Symbol: Tg(SOD1*G93A)^dl1Gur
Name: transgene insertion dl 1, Mark E Gurney
MGI accession ID: MGI:2671112
Generation method: Transgenic
Attributes: Inserted expressed sequence; Humanized sequence
Marker symbol: Tg(SOD1*G93A)^dl1Gur
Marker name: transgene insertion dl 1, Mark E Gurney
Chromosome: 12
Strain of origin: (C57BL/6 x SJL)F1
Expressed genes: SOD1,superoxide dismutase 1,human
Molecular note: 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.
General note: 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.