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
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  SOD1 (6647)

  Sod1	(MGI:98351)

• Mutation details: A 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)

Disease models

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: 51 reference(s)