Article
Assessment of transthyretin instability in patients with wild-type transthyretin amyloid cardiomyopathy.
Scientific reports - 3 Sept 2024
Iino Takuya, Nagao Manabu, Tanaka Hidekazu, Yoshikawa Sachiko, Asakura Junko, Nishimori Makoto, Shinohara Masakazu, Harada Amane, Watanabe Shunsuke, Ishida Tatsuro, Hirata Ken-Ichi, Toh Ryuji
Abstract excerpt
The pathophysiology of variant transthyretin (TTR) amyloidosis (ATTRv) is associated with destabilizing mutations in the TTR tetramer. However, why TTR with a wild-type genetic sequence misfolds and aggregates in wild-type transthyretin amyloidosis (ATTRwt) is unknown. Here, we evaluate kinetic TTR stability with a newly developed ELISA system in combination with urea-induced protein denaturation. Compared with...
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