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Transgene Detail
Symbol: Tg(SOD1*G93A)dl1Gur
Name: transgene insertion dl 1, Mark E Gurney
MGI ID: MGI:2671112
Synonyms: G1L, G93A SOD1, mSOD1-Tg, TgNSOD1-G93A, TgN(SOD1-G93A)1Gurdl, TgN(SOD1-G93A)1Gurdl, TgN[SOD1-G93A]dl1Gur, Tg(SOD1-G93A)dl1Gur
Transgene: Tg(SOD1*G93A)dl1Gur  Location: unknown  Genetic Position: Chr12, Syntenic
Strain of Origin:  (C57BL/6 x SJL)F1
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
Tg(SOD1*G93A)dl1Gur expresses 1 gene
Mutation detailsThis 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. (J:32665, J:106420)
View phenotypes and curated references for all genotypes (concatenated display).
Disease models
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Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
Carrying this Mutation:  Mouse Strains: 2 strains available      Cell Lines: 0 lines available
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.

Original:  J:106420 Zhang B, et al., Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation. J Cell Biol. 1997 Dec 1;139(5):1307-15
All:  62 reference(s)

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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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MGI 6.14
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