C Nediani, J Ruzzolini, A Romani, L Calorini - Antioxidants, 2019 - mdpi.com
Growing scientific literature data suggest that the intake of natural bioactive compounds plays a critical role in preventing or reducing the occurrence of human chronic non …
The mechanisms underlying transthyretin‐related amyloidosis in vivo remain unclear. The abundance of the 49–127 transthyretin fragment in ex vivo deposits suggests that a …
J Dobson, A Kumar, LF Willis, R Tuma… - Proceedings of the …, 2017 - National Acad Sciences
Relative to other extrinsic factors, the effects of hydrodynamic flow fields on protein stability and conformation remain poorly understood. Flow-induced protein remodeling and/or …
The protein homeostasis (proteostasis) system encompasses the cellular processes that regulate protein synthesis, folding, concentration, trafficking and degradation. In the case of …
PP Mangione, R Porcari, JD Gillmore… - Proceedings of the …, 2014 - National Acad Sciences
The Ser52Pro variant of transthyretin (TTR) produces aggressive, highly penetrant, autosomal-dominant systemic amyloidosis in persons heterozygous for the causative …
Abstract Dialysis-related amyloidosis (DRA), a serious complication among long-term hemodialysis patients, is caused by amyloid fibrils of β2-microglobulin (β2m). Although high …
A Gershenson, LM Gierasch, A Pastore… - Nature chemical …, 2014 - nature.com
Evolutionary pressure for protein function leads to unavoidable sampling of conformational states that are at risk of misfolding and aggregation. The resulting tension between …
M Stoppini, V Bellotti - Journal of biological chemistry, 2015 - ASBMB
β 2-Microglobulin is responsible for systemic amyloidosis affecting patients undergoing long- term hemodialysis. Its genetic variant D76N causes a very rare form of familial systemic …
JC Saunders, LM Young, RA Mahood… - Nature chemical …, 2016 - nature.com
Protein aggregation underlies an array of human diseases, yet only one small-molecule therapeutic targeting this process has been successfully developed to date. Here, we …