| Primary Identifier | MGI:3629226 | Allele Type | Transgenic |
| Attribute String | Conditional ready, Humanized sequence, Inserted expressed sequence | Gene | Tg(SOD1*G37R)1Dwc |
| Strain of Origin | (C57BL/6J x C3H/HeJ)F2 | Is Recombinase | false |
| Is Wild Type | false |
| description | 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. |
| molecularNote | 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. |
