Localization of mutant SLC37A4
(A) Subcellular fractionations of extracts from control C9 and edited lines C21 and C71 showing SLC37A4 protein is absent from the GM130 Golgi-containing fractions, but with similar fractionation pattern as the ER marker, calnexin. Subcellular fractionations were performed with three biological replicates via the Nycodenz gradient method, and representative images are shown.
(B) Immunofluorescence staining of Huh7 control and edited cells showing localization of SLC37A4 with the ER marker, KDEL (upper panel), the Golgi marker, GM130 (middle panel), and the ER exit site marker, SEC31 (lower panel).
(C) Immunofluorescence staining for the ER marker, KDEL, and SLC37A4 in iPSC-derived hepatocytes from control and P7. Control (upper panel) showed strong colocalization (r = 0.98), while P7 (lower panel) showed reduced colocalization (r = 0.41).