Figure 6
Properties of ATP7B mutants located within the N-domain. (A) Multiple sequence alignment showing conservation of Gly1061 (G1061) and Gly1101 (G1101). (B) Location of G1061 and G1101 (shown in red) in the N-domain (in grey). The conserved SEHPL motif containing the common H1069Q mutation is in green. The actuator (A)-domain is in pink. (C) Localization of the G1061E ATP7B mutant in HEK293 cells under low and high Cu conditions. HEK293 cells were transfected (12 h) with WT or G1061E mutant 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).Endoplasmic reticulum (ER) marker, Calnexin, in red and nuclei (DAPI) in blue. Co-localization is indicated in yellow. Green signals are from GFP fluorescence.
