FVB-Tg(Sod1*G86R)M1Jwg/J

Stock number: 005110
Strain synonyms: M1
Research areas: Neurobiology Research

Description

These mice express a transgene containing a missense mutation of the Sod1 gene that confers a progressive loss of motor function.

Detailed description

These transgenic mice express the missense mutant mouse Sod1 under the control of the endogenous promoter. The missense mutation is a point mutation in exon 4 resulting in a glycine-86 to arginine substitution,which corresponds to amino acid position 86 in the human SOD1 protein. Transgene expression is detected by RT-PCR analysis of brain, spinal cord, and other tissues. Onset of progressive loss of motor function begins at 3-4 months of age with hind limb spastic paralysis and muscle wasting. Transgenic mice do not survive beyond 4 months of age. Histological analysis of spinal cord ventral horns, brain stem and neocortex reveals neurodegeneration and abnormal neurites. Affected mice do not exhibit diminished SOD1 activity. This mutant mouse strain may be useful in studies of amyotrophic lateral sclerosis.

Development

A transgenic construct containing 1.5kb sequence of the modified mouse Sod1 gene with the missense mutation in exon 4, and flanking regions was injected into fertilized FVB/N mouse eggs. Founder animals were bred to wildtype FVB/N mice.

Allele

Symbol: Tg(Sod1*G86R)M1Jwg
Name: transgene insertion M1, Jon W Gordon
MGI accession ID: MGI:2183736
Generation method: Transgenic
Attributes: Inserted expressed sequence; Humanized sequence
Marker symbol: Tg(Sod1*G86R)M1Jwg
Marker name: transgene insertion M1, Jon W Gordon
Chromosome: UN
Strain of origin: FVB/N
Expressed genes: Sod1,superoxide dismutase 1, soluble,mouse, laboratory
Molecular note: The entire mouse Sod1 gene with an engineered point mutation in exon 4 resulting in a glycine to arginine substitution at residue 86 (G86R) was used as the transgene. Mutation G86R corresponds to the SOD1 G86R mutation found in some cases of human amyotrophic lateral sclerosis (ALS) patients. RT-PCR analysis detected expression of the transgene in brain, spinal cord, and other tissues. The missense mutation is a point mutation in exon 4 resulting in a glycine-86 to arginine substitution, which corresponds to amino acid position 86 in the human SOD1 protein. Transgenics exhibit widespread high expression of the transgene and do not exhibit diminished SOD1 activity. .