Fig. 4.

Stabilization of V122I-TTR in serum from two patients with FAC against acid-mediated denaturation in the presence of AG10 and tafamidis. Stabilization effect of tafamidis and AG10 on serum from a heterozygous patient with FAC (heterozygous V122I/WT-TTR, African American, male, age 56 y) at 0 h (A and B) and after 72 h (C and D). (E and F) Stabilization effect of tafamidis and AG10 on serum from a homozygous patient with FAC (homozygous V122I-TTR, Caucasian, female, age 62 y). Sample processing and quantification was performed as described in Fig. 3. IgG was analyzed by using IRdye800 goat anti-human IgG. Error bars represent SEM of three replicates.