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| Nomenclature |
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Symbol:
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Tg(SOD1*G37R)1Dwc
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Name:
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transgene insertion 1, Don W Cleveland
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MGI ID: |
MGI:3629226 |
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Synonyms: |
LoxSOD1G37R |
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Transgene:
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Tg(SOD1*G37R)1Dwc
Location:
unknown
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Transgene
origin |
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Strain of Origin:
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(C57BL/6J x C3H/HeJ)F2
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Transgene
description |
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Transgene
Type: | |
Transgenic (random, expressed) |
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Mutation: | |
Insertion |
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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)
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Phenotypes
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View phenotypes for all genotypes (concatenated display).
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Disease models
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| Find Mice (IMSR) |
Mouse strains and cell lines available from the
International Mouse Strain Resource
(IMSR)
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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.
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| References |
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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
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All: |
7 reference(s)
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Analysis Tools
