BLOC1S3 and cancer: Current hypotheses for this cancer predisposition include specific alterations to the spectrum of translation due to defective ribosome biogenesis, a loss of translational fidelity due to selection of second-site suppressor mutations, selective pressure for p53 mutations due to the chronic p53 activity in such genotypes, and oxidative stress or metabolic reprogramming in Rp+/-cells (Sulima et al., 2019).