SPRTN and progeroid features-hepatocellular carcinoma predisposition syndrome: RJALS is caused by monogenic and biallelic mutations in SPRTN (DVC1), and a single missense mutation in a putative metalloprotease SprT domain (SPRTNY117C) is pathogenic and responsible for premature aging and liver cancer in humans (Lessel et al., 2014, Ramadan et al., 2016).