Figure 2.
Age-related penetrance of the GGGGCC hexanucleotide repeat expansion in the C9ORF72 gene based on an analysis of 603 mutant-gene carriers (n = 212 familial ALS patients, n = 234 sporadic ALS, n = 99 familial FTD, n = 53 sporadic FTD, and n = 5 neurologically normal controls). The age-related penetrance (i.e., the proportion of mutant-gene carriers with manifestations of the disease by a given age) rose steadily, from 10% in the <45 years-old group, to ~100% by the age of 80 years. The dotted lines represent the age at which 50% of the cohort developed symptoms.