In contrast, the FVB/NJ-Tg(C9orf72)500Lpwr/J mouse model expressing the full length mutated human C9orf72 gene including the 52 kb 5′ upstream region and the 19 kb 3′ downstream region of the gene did show progressive neurodegeneration and decreased survival which is also seen in ALS patients (Liu et al., 2016). Here, C9orf72 is linked to amyotrophic lateral sclerosis.