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Tg(SOD1*G85R)148Dwc
Transgene Detail
Summary
Symbol: Tg(SOD1*G85R)148Dwc
Name: transgene insertion 148, Don W Cleveland
MGI ID: MGI:2182171
Synonyms: 148-G85R, G85R, G85R line 148, G85R SOD1 line148, SOD1 G85R line 148, Tg(SOD1-G85R)148Dwc
Transgene: Tg(SOD1*G85R)148Dwc  Location: unknown  
Alliance: Tg(SOD1*G85R)148Dwc page
Transgene
origin
Strain of Origin:  Not Applicable
Transgene
description
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
 
Tg(SOD1*G85R)148Dwc expresses 1 gene
 
Mutation detailsA 12 kb genomic fragment of human SOD1 carrying a glycine to arginine substitution at amino acid 85 (G85R), a mutation associated with familial amyotrophic lateral sclerosis (ALS), was used as the transgene. (J:77600)
Phenotypes
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View phenotypes and curated references for all genotypes (concatenated display).
Disease models
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Expression
In Structures Affected by this Mutation: 2 anatomical structure(s)
Find Mice (IMSR)
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
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.
References
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
All:  52 reference(s)

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory