Figure 8

Molecular and cellular characteristics of the S1362A ATP7B mutant. (A) Multiple sequence alignment showing conservation of Ser1362. (B) Location of Ser1362 (red) in proximity to the invariant CPC motif (green) involved in Cu coordination within the transmembrane domain. (C) Localization of S1362A mutant ATP7B in HEK293 cells under low and high Cu conditions. HEK293 cells were transfected (12 h) with WT or S1362A mutant ATP7B GFP-ATP7B (green) and treated for 3 h with basal medium (top), medium containing 100 µM of CuCl2 (middle), or medium containing 25 µM of tetra-thiomolybdate (TTM) (lower panel). Trans-golgi network (TGN) marker, TGN46, in red and nuclei (DAPI) in blue. Co-localization is indicated in yellow. Green signals are from GFP fluorescence.