Tg(SOD1*H46R*H48Q)58Dbo
Transgene Detail

Summary

• Symbol: Tg(SOD1*H46R*H48Q)58Dbo
• Name: transgene insertion 58, David Borschelt
• MGI ID: MGI:3695884
• Transgene: Tg(SOD1*H46R*H48Q)58Dbo Location: unknown
• Alliance: Tg(SOD1*H46R*H48Q)58Dbo page

Transgene origin

• Strain of Origin: (C3H/HeJ x C57BL/6J)F2

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(SOD1*H46R*H48Q)58Dbo expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  SOD1 (6647)

  Sod1	(MGI:98351)

• Mutation details: A 12kb genomic fragment containing the entire human SOD1 gene was mutated so that the histidine residue at position 46 was replaced by arginine and the histidine at position 48 was replaced by glutamine in the encoded protein. This line is the representative line of 10 that developed motor neuron disease before 1 year of age. Lines 73, 67, and 58 show identical pathological changes. (J:119631)

Disease models

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 0 strains available Cell Lines: 0 lines available

References

• Original: J:119631 Wang J, et al., Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiol Dis. 2002 Jul;10(2):128-38
• All: 8 reference(s)