Tg(Prnp-Immt/SOD1*G93A)7Gmnf
Transgene Detail

Summary

• Symbol: Tg(Prnp-Immt/SOD1*G93A)7Gmnf
• Name: transgene insertion 7, Giovanni Manfredi
• MGI ID: MGI:5575878
• Synonyms: mito-G93ASOD1, mito-SOD1G93A, Prp-mito-G93ASOD1
• Transgene: Tg(Prnp-Immt/SOD1*G93A)7Gmnf Location: unknown
• Alliance: Tg(Prnp-Immt/SOD1*G93A)7Gmnf page

Transgene origin

• Strain of Origin: (C57BL/6 x CBA)F1

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(Prnp-Immt/SOD1*G93A)7Gmnf expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  SOD1 (6647)

  Sod1	(MGI:98351)

• Mutation details: The mito-G93ASOD1 fusion protein contains a mouse mitofilin (Immt) cDNA sequence encoding the first 187 amino acids (including the mitochondrial targeting presequence, MMP cleavage site and transmembrane domain) fused in-frame to the N-terminus of a human Cu,Zn superoxide dismutase cDNA sequence harboring the G93A mutation (SOD1) associated with amyotrophic lateral sclerosis (ALS). The SOD1 start codon was removed and substituted with an in-frame 6 nt DNA linker. This mito-G93ASOD1 sequence was inserted between exon 2 and exon 3 of mouse prion protein (PrP or Prnp) gene at a unique XhoI site in the MoPrP.Xho plasmid vector. The founder line with the highest expression level (founder line 7) was characterized. (J:177846)

Disease models

Expression

In Structures Affected by this Mutation:

8 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

References

• Original: J:177846 Igoudjil A, et al., In Vivo Pathogenic Role of Mutant SOD1 Localized in the Mitochondrial Intermembrane Space. J Neurosci. 2011 Nov 2;31(44):15826-37
• All: 2 reference(s)