In both human patients and mouse models carrying a C9orf72 mutation, leading to deposition of polyGA-inclusion bodies and a phenotype similar to amyotrophic lateral sclerosis with frontotemporal dementia, co-deposition of MLF2 with the polyGA deposits has been demonstrated [28] Similarly, in a Drosophila model of Huntington’s disease, spatial proximity of dMLF2 to toxic poly(Q) deposits has been shown and a protective role of MLF2 was suggested [29]. This evidence concerns the gene C9orf72 and juvenile Huntington disease.