Analysis of the interactome of RPS19 mutants

Ribosomal protein S19 (RPS19) is a 16-kDa protein found mainly as a component of the ribosomal 40 S subunit. Its mutations are responsible for Diamond Blackfan anemia, a congenital disease characterized by defective erythroid progenitor maturation. To clarify the function of RPS19, we compare the interactome of RPS19 wilde type and their two mutant proteins: RPS19-R62W and RPS19- R101H. Mutations R62W and R101H impair RPS19 ability to associate with the ribosome. We found striking differences between the interactomes of wild type and mutant RPS19 proteins. In particular, mutations abolish interactions with proteins having splicing, translational and helicase activity, thus confirming the role of RPS19 in RNA processing/metabolism and translational control.