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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Autores principales:	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
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/a4b751bc357945ceb32e93795210aa97
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spelling	oai:doaj.org-article:a4b751bc357945ceb32e93795210aa972021-12-02T11:44:56ZNAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients10.1038/s41467-021-22120-42041-1723https://doaj.org/article/a4b751bc357945ceb32e93795210aa972021-03-01T00:00:00Zhttps://doi.org/10.1038/s41467-021-22120-4https://doaj.org/toc/2041-1723HCCAA 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-carrying subjects.Michael E. MarchAlvaro Gutierrez-UzquizaAsbjorg Osk SnorradottirLeticia S. MatsuokaNoelia Fonseca BalvisThorgeir GestssonKenny NguyenPatrick M. A. SleimanCharlly KaoHelgi J. IsakssonBirkir Thor BragasonElias OlafssonAstridur PalsdottirHakon HakonarsonNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q 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 NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients
description	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-carrying subjects.
format	article
author	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
author_facet	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
author_sort	Michael E. March
title	NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients
title_short	NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients
title_full	NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients
title_fullStr	NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients
title_full_unstemmed	NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients
title_sort	nac blocks cystatin c amyloid complex aggregation in a cell system and in skin of hccaa patients
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/a4b751bc357945ceb32e93795210aa97
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NAC blocks Cystatin C amyloid complex aggregation in a cell system and in skin of HCCAA patients

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