Official websites use .gov
A
.gov website belongs to an official
government organization in the United States.
Secure .gov websites use HTTPS
A lock (
) or https:// means you've safely
connected to the .gov website. Share sensitive
information only on official, secure websites.
ERS pathway was activated in COL4A5 mutant fibroblasts. (A) Protein expression of the ER stress markers Bip, ATF6, p50-ATF6, IRE1α, p-IRE1α, PERK, and p-PERK. β-tubulin was used as an equal loading control (Supplementary Figure 2). (B–H) Representative densitometric analysis of western blots of these proteins. Data are means ± SEM. n = 3 (*p < 0.05; **p < 0.01; ***p < 0.001; WT, control; MM, missense mutation; SM, splicing mutation; FM, frameshift mutation).
