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Transgene Detail
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  
Strain of Origin:  Not Applicable
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)
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Carrying this Mutation:  Mouse Strains: 1 strain available      Cell Lines: 0 lines available
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.
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:  37 reference(s)

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MGI 6.07
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