Tg(SOD1*G93A)1Gur
Transgene Detail

Nomenclature

Symbol:	Tg(SOD1*G93A)1Gur
Name:	transgene insertion 1, Mark E Gurney
MGI ID:	MGI:2183719
Synonyms:	G1H, G93A, G93A+, G93AGur^dl, G93A SOD1, G93A-SOD1, (G93A)Tg+, hSOD1G93A, SOD1^G93A, SOD1 G93A, SOD1 Tg, Tg(G93A-SOD1)1Gur, TgN(SOD1-G93A)1Gur, TgN[SOD1-G93A]1Gur, Tg(SOD1-G93A)1Gur
Transgene:	Tg(SOD1*G93A)1Gur  Location: unknown  Genetic Position: Chr12, cytoband E

Presence of neurofilament spheroids in the spinal cord of an 82-day old Tg(SOD1)2Gur/0 mouse

Show the 5 phenotype image(s) involving this allele.

Transgene origin

Strain of Origin:

(C57BL/6 x SJL)F1

Transgene description

Transgene Type:		Transgenic (Inserted expressed sequence)
Mutation:		Insertion
		Mutation details: This transgenic subline (designated G1H in J:76718) is derived from the G1 parental transgenic line (originally described in J:32665). This line carries a 40% expansion in transgene copy number compared to the original G1 line (described in J:32665, in MGI as Tg(SOD1*G93A)2Gur). The transgene construct is composed of the human SOD1 gene carrying a glycine to alanine transition at position 93 (G93A). The G93A mutation does not alter the activity of the protein. This line carries a high copy number. (J:32665, J:76718)

Disease models

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Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
Carrying this Mutation:	Mouse Strains: 3 strains available      Cell Lines: 0 lines available

Notes

This line, G1H, was derived from the original G1 line (now designated Tg(SOD1*G93A)2Gur) reported in J:32665.

Transgenic mice on a background that involves C57BL/6 and SJL express high levels of the transgene with a 4-fold increase in SOD activity, and exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans. Hemizygous transgenic mice become paralyzed in one or more limbs and have a life span of approximately 19-23 weeks. Paralysis is due to loss of motor neurons from the spinal cord.

References

Original:	J:76718 Tu PH, et al., Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. Proc Natl Acad Sci U S A. 1996 Apr 2;93(7):3155-60
All:	396 reference(s)