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| Nomenclature |
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Symbol:
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Tg(SOD1*G85R)74Dwc
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Name:
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transgene insertion 74, Don W Cleveland
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MGI ID: |
MGI:2182172 |
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Synonyms: |
74-G85R, Tg(SOD1-G85R)74Dwc |
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Transgene:
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Tg(SOD1*G85R)74Dwc
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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Mutation details: A 12 kb genomic fragment of human SOD1 carrying the glycine to arginine substitution at amino acid 85 (G85R) associated with familial amyotrophic lateral sclerosis (ALS) was used as the transgene. (J:77600)
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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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Carrying this Mutation: |
Mouse Strains: 0 strains available
Cell Lines: 0 lines available |
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Notes |
Transgenic animals initially exhibit hindlimb weakness that spreads with rapid progression to forelimbs and results in muscle atrophy and complete paralysis within 2 weeks. Founders 148-G85R and 74-G85R are the highest and lowest expressing transgenic lines, respectively, and differ in onset of clinical symptoms, presumably due to gene dosage. Founder 148-G85R exhibits muscle weakness at 8-10 months of age whereas founder 74-G85R exhibits the phenotype 12-14 months of age. Despite the difference in disease onset both founders show the same rate of disease progression after appearance of initial symptoms.
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| References |
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Original: |
J:77600
Bruijn LI et al.,
"ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions."
Neuron 1997 Feb;18(2):327-38
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All: |
1 reference(s)
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Analysis Tools
