Co-immunoprecipitation (Co-IP) of Myc-USP11 with SFB-SPRTN full-length (FL) or ΔSprT, ΔSH, ΔPIP, ΔUBZ, E112A catalytic inactive, and Y117C (SPRTN mutation identified in RJALS patients) mutant constructs showed that SPRTN-USP11 interaction was lost with deletion of the N-terminal SprT domain of SPRTN (Fig. 2A). The gene discussed is USP11; the disease is progeroid features-hepatocellular carcinoma predisposition syndrome.