Tg(Prnp-FUS*R495X)78Ljh
Transgene Detail

Summary

• Symbol: Tg(Prnp-FUS*R495X)78Ljh
• Name: transgene insertion 78, Lawrence Hayward
• MGI ID: MGI:5621045
• Synonyms: FUS^R495X, line PX78
• Transgene: Tg(Prnp-FUS*R495X)78Ljh Location: unknown
• Alliance: Tg(Prnp-FUS*R495X)78Ljh page

Transgene origin

• Strain of Origin: (C57BL/6 x SJL)F2

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(Prnp-FUS*R495X)78Ljh expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  FUS (2521)

  Fus	(MGI:1353633)

  human fused in sarcoma R495X truncation mutant (hFUS*R495X) that is associated with amyotrophic lateral sclerosis (ALS)

• Mutation details: The transgenic construct contains a cDNA sequence encoding the human fused in sarcoma R495X truncation mutant (hFUS*R495X) associated with amyotrophic lateral sclerosis (ALS). The cDNA sequence was inserted between exon 2 and exon 3 of mouse prion protein (PrP or Prnp) gene. The mutation abrogates a putative nuclear localization signal at the C-terminus of FUS, resulting in significant hFUS mislocalization within the cytoplasm. Line PX78 is identified with 10-12 copies of the transgene and results in a 3-5-fold FUS overexpression. (J:218094)

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:218094 Tibshirani M, et al., Cytoplasmic sequestration of FUS/TLS associated with ALS alters histone marks through loss of nuclear protein arginine methyltransferase 1. Hum Mol Genet. 2015 Feb 1;24(3):773-86
• All: 3 reference(s)