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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
description |
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Transgene
Type: | |
Transgenic (random, expressed) |
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Mutation: | |
Insertion |
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A pair of 34 bp loxP sites were cloned to each end of the human SOD1 carrying the G37R mutation. (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
