NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients

HCCAA is a dominantly inherited disease which causes brain hemorrhages as a result of mutant cystatin C aggregation in carriers. Here, the authors show that n- acetyl cysteine can prevent aggregation of mutant protein in a cell model system and reverse protein deposition in the skin of mutation-carr...

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Bibliographic Details
Main Authors: Michael E. March, Alvaro Gutierrez-Uzquiza, Asbjorg Osk Snorradottir, Leticia S. Matsuoka, Noelia Fonseca Balvis, Thorgeir Gestsson, Kenny Nguyen, Patrick M. A. Sleiman, Charlly Kao, Helgi J. Isaksson, Birkir Thor Bragason, Elias Olafsson, Astridur Palsdottir, Hakon Hakonarson
Format: Article
Language:English
Published: Nature Publishing Group 2021-03-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-021-22120-4