USP54 and cholestasis: Mapping all cholestasis-related single amino acid substitutions in USP53 (Fig. 2a) on a homology model based on our USP54 structure illustrated that several substituted residues are either part of the above-described zinc fingers (explaining the reduced stability and impaired catalytic activity of the H132Y protein) or are located around the active site (Extended Data Fig. 8a–e).