Tg(Prnp-FUS*R495X)78Ljh

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)