TANGO1S is required for collagen VII export from the ER. (A) HA tag alone, TANGO1S-HA, or TANGO1L-HA were expressed in 293T cells and immunoprecipitated with anti-HA antibody. Immunoprecipitants were further incubated with lysate prepared from 293T cells stably expressing FLAG-collagen VII. The beads were washed and subjected to SDS–PAGE, followed by Western blotting with anti-FLAG or anti-HA antibodies. (B) HSC-1 cells were transfected with the indicated siRNA. After 72 h, cells were fixed and stained with anti–collagen VII antibody. Scale bar, 10 μm. (C) HSC-1 cells were transfected with the indicated siRNA(s) and stained with anti–collagen VII antibody. The collagen VII immunofluorescence signal per cell was quantified (arbitrary units [A.U.]). Error bars represent mean ± SEM. **p < 0.005 compared with control siRNA. For cells treated with control siRNA, n = 92; with TANGO1L (#1), n = 93; with TANGO1L (#2), n = 102; with TANGO1S (#1), n = 109; with TANGO1S (#2), n = 84; with TANGO1L (#1) and TANGO1S (#1), n = 112; and with TANGO1L (#2) and TANGO1S (#2), n = 120.