Mutations of proteins involved in maintenance and repair of telomeres are responsible for telomeropathies [8,9]: a spectrum of progressive genetic diseases exemplified in the most severe cases by dyskeratosis congenita (DKC), whose common autosomal recessive form is caused by mutations in DKC1. They are degenerative and age-dependent diseases, characterized by premature senescence of the stem cell compartment, determining increased risk of organ failure and cancer, with possible involvement of the hematopoietic compartment, lungs, mucous membranes, skin, and also the liver. Here, DKC1 is linked to dyskeratosis congenita.