Mutations directly implicated as causing DC have been identified in the genes DKC1, TERC, TERT, NOP10, NHP2 and TINF2. The first five of these genes all encode components of the telomerase holoenzyme while TINF2 encodes a component of the telomere shelterin complex (Fig. 3). This evidence concerns the gene NOP10 and dyskeratosis congenita.