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Tg(SOD1*G37R)1Dwc
Transgene Detail
Nomenclature
Symbol: Tg(SOD1*G37R)1Dwc
Name: transgene insertion 1, Don W Cleveland
MGI ID: MGI:3629226
Synonyms: LoxSOD1G37R
Transgene: Tg(SOD1*G37R)1Dwc  Location: unknown  
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
 
Mutation detailsThe 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)
Phenotypes
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Disease models
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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:  10 reference(s)

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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
07/21/2015
MGI 5.22
The Jackson Laboratory