Tg(SOD1*G93A)^dl1Gur
Transgene Detail

Summary

• 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)1Gur^dl, TgN(SOD1-G93A)1Gurdl, TgN[SOD1-G93A]^dl1Gur, Tg(SOD1-G93A)^dl1Gur
• Transgene: Tg(SOD1*G93A)^dl1Gur Location: unknown Genetic Position: Chr12, Syntenic
• Alliance: Tg(SOD1*G93A)^dl1Gur page

Transgene origin

• Strain of Origin: (C57BL/6 x SJL)F1

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(SOD1*G93A)^dl1Gur expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  SOD1 (6647)

  Sod1	(MGI:98351)

• Mutation details: 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. (J:32665, J:106420)

Disease models

Expression

In Structures Affected by this Mutation:

2 anatomical structure(s)

Find Mice (IMSR)

• 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

Notes

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.

References

• 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: 79 reference(s)