C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD (original) (raw)

“…Another formal test of loss- vs. gain-of-function involves the use of C9orf72 knockout mice and some of the recently described viral-mediated and BAC transgenic c9FTD/ALS models (Chew et al, 2015; O’Rourke et al, 2015; Peters et al, 2015). These models employ expression of human C9orf72 transgenes harboring various GGGGCC repeat lengths either via adeno-associated virus mediated somatic transgenesis (Chew et al, 2015) or in transgenic mice generated from a bacterial artificial chromosomes (BAC) that expresses a fragment of human C9orf72 containing an expanded hexanucleotide repeat (O’Rourke et al, 2015; Peters et al, 2015) or the full length C9orf72 gene harboring an expanded repeat (O’Rourke et al, 2015).…”