Treatment with ROS detoxifying gold quantum clusters alleviates the functional decline in a mouse model of Friedreich ataxia.

Animals Disease Models, Animal Friedreich Ataxia Gold Humans Mice Reactive Oxygen Species Trinucleotide Repeat Expansion

Journal

Science translational medicine
ISSN: 1946-6242
Titre abrégé: Sci Transl Med
Pays: United States
ID NLM: 101505086

Informations de publication

Date de publication:
18 08 2021
Historique:
received: 04 08 2020
accepted: 15 06 2021
entrez: 19 8 2021
pubmed: 20 8 2021
medline: 26 8 2021
Statut: ppublish

Résumé

Friedreich ataxia (FRDA) is caused by the reduced expression of the mitochondrial protein frataxin (FXN) due to an intronic GAA trinucleotide repeat expansion in the

Identifiants

DOI: 10.1126/scitranslmed.abe1633 PMID: 34408077
pubmed: 34408077
pii: 13/607/eabe1633
doi: 10.1126/scitranslmed.abe1633
pii:
doi:

Substances chimiques

Reactive Oxygen Species 0
Gold 7440-57-5

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : NINDS NIH HHS
ID : R01 NS079965
Pays : United States

Informations de copyright

Copyright © 2021 The Authors, some rights reserved; exclusive licensee American Association for the Advancement of Science. No claim to original U.S. Government Works.

Auteurs

Chiara Villa (C)

Stem Cell Laboratory, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Centro Dino Ferrari, Via F. Sforza 35, 20122 Milano, Italy.
ORCID: 0000-0002-3002-3372

Mariella Legato (M)

Stem Cell Laboratory, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Centro Dino Ferrari, Via F. Sforza 35, 20122 Milano, Italy.

Alessandro Umbach (A)

Department of Molecular Biotechnology and Health Science, University of Turin, Via Nizza, 52 10126 Torino, Italy.
ORCID: 0000-0003-3681-3110

Chiara Riganti (C)

Department of Oncology, University of Turin, Via Santena 5/bis, 10126 Torino, Italy.
ORCID: 0000-0001-9787-4836

Rebecca Jones (R)

Department of Molecular Biotechnology and Health Science, University of Turin, Via Nizza, 52 10126 Torino, Italy.
ORCID: 0000-0003-0427-3623

Beatrice Martini (B)

Stem Cell Laboratory, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Centro Dino Ferrari, Via F. Sforza 35, 20122 Milano, Italy.

Marina Boido (M)

Department of Neuroscience "Rita Levi Montalcini", Neuroscience Institute Cavalieri Ottolenghi, University of Turin, Regione Gonzole 10, Orbassano,10043 Torino, Italy.
ORCID: 0000-0003-1211-1552

Claudio Medana (C)

Department of Molecular Biotechnology and Health Science, University of Turin, Via Nizza, 52 10126 Torino, Italy.
ORCID: 0000-0002-9772-6814

Irene Facchinetti (I)

Department of Material Science, University of Milano Bicocca, Via R. Cozzi 55, 20125 Milano, Italy.
ORCID: 0000-0001-7178-5368

Dario Barni (D)

Department of Material Science, University of Milano Bicocca, Via R. Cozzi 55, 20125 Milano, Italy.
ORCID: 0000-0001-9571-2956

Milena Pinto (M)

Department of Neurology, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
ORCID: 0000-0001-9202-1527

Tania Arguello (T)

Department of Neurology, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
ORCID: 0000-0003-4761-6593

Marzia Belicchi (M)

Stem Cell Laboratory, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Centro Dino Ferrari, Via F. Sforza 35, 20122 Milano, Italy.
ORCID: 0000-0001-7916-2325

Gigliola Fagiolari (G)

Neuromuscular and Rare Diseases Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milan, Italy.

Carla Liaci (C)

Department of Molecular Biotechnology and Health Science, University of Turin, Via Nizza, 52 10126 Torino, Italy.
ORCID: 0000-0003-4508-516X

Maurizio Moggio (M)

Neuromuscular and Rare Diseases Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milan, Italy.

Riccardo Ruffo (R)

Department of Material Science, University of Milano Bicocca, Via R. Cozzi 55, 20125 Milano, Italy.
ORCID: 0000-0001-7509-7052

Carlos T Moraes (CT)

Department of Neurology, University of Miami Miller School of Medicine, Miami, FL 33136, USA.

Angelo Monguzzi (A)

Department of Material Science, University of Milano Bicocca, Via R. Cozzi 55, 20125 Milano, Italy.
ORCID: 0000-0001-9768-4573

Giorgio R Merlo (GR)

Department of Molecular Biotechnology and Health Science, University of Turin, Via Nizza, 52 10126 Torino, Italy.

Yvan Torrente (Y)

Stem Cell Laboratory, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Centro Dino Ferrari, Via F. Sforza 35, 20122 Milano, Italy. yvan.torrente@unimi.it.
ORCID: 0000-0002-2705-3984

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Classifications MeSH

questionsmedicales.fr › Eucaryotes › Animaux › Treatment with ROS detoxifying gold quantum...
questionsmedicales.fr › Techniques d'investigation › Modèles théoriques › Modèles biologiques › Modèles animaux de maladie humaine › Treatment with ROS detoxifying gold quantum...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Maladies mitochondriales › Ataxie de Friedreich › Treatment with ROS detoxifying gold quantum...
questionsmedicales.fr › Produits chimiques inorganiques › Métaux › Métaux lourds › Or › Treatment with ROS detoxifying gold quantum...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Treatment with ROS detoxifying gold quantum...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Treatment with ROS detoxifying gold quantum...
questionsmedicales.fr › Produits chimiques inorganiques › Composés de l'oxygène › Espèces réactives de l'oxygène › Treatment with ROS detoxifying gold quantum...
questionsmedicales.fr › Phénomènes génétiques › Mutagenèse › Expansion de séquence répétée de l'ADN › Expansion de trinucléotide répété › Treatment with ROS detoxifying gold quantum...