Mutations in ELA2 lead to cytoplasmic accumulation of MT-HNE proteins in transiently transfected HeLa cells. Subcellular localization of WT- or MT-HNE protein in HeLa cells. Cells were labeled with anti-HNE (green) and anticalnexin (ER marker; red), antigiantin (Golgi marker; red), and anti–lamp-3 (lysosomal marker; red). TO-PRO-3 was used to counterstain the nuclei (blue). Yellow displays colocalization of HNE protein with organelle-specific markers (Figures S1-S5).