Tg(SOD1*G37R)1Dwc
Transgene Detail

Summary

• Symbol: Tg(SOD1*G37R)1Dwc
• Name: transgene insertion 1, Don W Cleveland
• MGI ID: MGI:3629226
• Synonyms: LoxSOD1^G37R
• Transgene: Tg(SOD1*G37R)1Dwc Location: unknown
• Alliance: Tg(SOD1*G37R)1Dwc page

Transgene origin

• Strain of Origin: (C57BL/6J x C3H/HeJ)F2

Transgene description

• Transgene Type: Transgenic (Conditional ready, Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(SOD1*G37R)1Dwc expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  SOD1 (6647)

  Sod1	(MGI:98351)

• Mutation details: The SOD1*G37R transgene was designed with the entire human "floxed"-superoxide dismutase 1, soluble (SOD1) gene, modified to harbor the SOD1*G37R mutation, driven by its endogenous human promoter. The expressed protein is characterized as an enzymatically active, "gain of adverse function" mutation. When transgenic mice are bred to mice expressing tissue-specific Cre recombinase, the resulting offspring will have the floxed-SOD1*G37R sequence deleted in the cre-expressing tissues. (J:109131)

Disease models

Expression

In Structures Affected by this Mutation:

1 anatomical structure(s)

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 1 strain available Cell Lines: 0 lines available

Notes

Mice develop fatal progressive motorneuron disease, including weight loss from denervation-induced muscle atrophy and paralysis. The highest expressing line reached end stage disease between 8.5 and 11 months. No human SOD1 was expressed in progeny from transgenic females that also expressed a germ line Cre transgene. The effects of mutant SOD1 within motorneurons was assessed by mating human mutant SOD1-expressing transgenic mice with mice expressing Cre under control of the Islet-1 promoter to remove expression from motorneurons specifically.

References

• Original: J:109131 Boillee S, et al., Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia. Science. 2006 Jun 2;312(5778):1389-92
• All: 27 reference(s)