Oxidative Stress in Transthyretin-Mediated Amyloidosis: An Exploratory Study

Marco Fiore; Chiara Cambieri; Laura Libonati; Federica Moret; Edoardo D’Andrea; Maria Grazia Di Certo; Claudio Passananti; Francesca Gabanella; Nicoletta Corbi; Matteo Garibaldi; Cristina Chimenti; Maria Alfarano; Giampiero Ferraguti; Silvia Francati; Maurizio Inghilleri; Marco Ceccanti

doi:10.3390/antiox13080998

Antioxidants (Aug 2024)

Oxidative Stress in Transthyretin-Mediated Amyloidosis: An Exploratory Study

Marco Fiore,
Chiara Cambieri,
Laura Libonati,
Federica Moret,
Edoardo D’Andrea,
Maria Grazia Di Certo,
Claudio Passananti,
Francesca Gabanella,
Nicoletta Corbi,
Matteo Garibaldi,
Cristina Chimenti,
Maria Alfarano,
Giampiero Ferraguti,
Silvia Francati,
Maurizio Inghilleri,
Marco Ceccanti

Affiliations

Marco Fiore: CNR-Institute of Biochemistry and Cell Biology, Via Ercole Ramarini 32, 00015 Monterotondo, Italy
Chiara Cambieri: Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell’Università 30, 00185 Rome, Italy
Laura Libonati: Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell’Università 30, 00185 Rome, Italy
Federica Moret: Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell’Università 30, 00185 Rome, Italy
Edoardo D’Andrea: Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell’Università 30, 00185 Rome, Italy
Maria Grazia Di Certo: CNR-Institute of Biochemistry and Cell Biology, Via Ercole Ramarini 32, 00015 Monterotondo, Italy
Claudio Passananti: CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy
Francesca Gabanella: CNR-Institute of Biochemistry and Cell Biology, Via Ercole Ramarini 32, 00015 Monterotondo, Italy
Nicoletta Corbi: CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy
Matteo Garibaldi: Department of Neuroscience, Mental Health, and Sensory Organs (NESMOS), Sant’Andrea Hospital, Sapienza University, 00189 Rome, Italy
Cristina Chimenti: Department of Clinical, Internal, Anesthesiologist and Cardiovascular Sciences, Sapienza University of Rome, 00185 Rome, Italy
Maria Alfarano: Department of Clinical, Internal, Anesthesiologist and Cardiovascular Sciences, Sapienza University of Rome, 00185 Rome, Italy
Giampiero Ferraguti: Department of Experimental Medicine, Sapienza University of Rome, 00185 Roma, Italy
Silvia Francati: Department of Experimental Medicine, Sapienza University of Rome, 00185 Roma, Italy
Maurizio Inghilleri: Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell’Università 30, 00185 Rome, Italy
Marco Ceccanti: Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell’Università 30, 00185 Rome, Italy

DOI: https://doi.org/10.3390/antiox13080998
Journal volume & issue: Vol. 13, no. 8
p. 998

Abstract

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Transthyretin-mediated amyloidosis (ATTR) is a systemic disease with protein precipitation in many tissues, mainly the peripheral nerve and heart. Both genetic (ATTRv, “v” for variant) and wild-type (ATTRwt) forms are known. Beyond the steric encumbrance, precipitated transthyretin seems to have a toxic effect. In this study carried out in men, we recruited 15 ATTRv patients, 7 ATTRv asymptomatic carriers, 14 ATTRwt patients and 10 young and 13 old healthy controls to evaluate the oxidative stress using FORD (Free Oxygen Radicals Defense) and FORT (Free Oxygen Radicals Test) analyses. ATTRv patients showed reduced FORD compared to ATTRwt and ATTRv asymptomatic carriers. FORD independently predicted the disease stage, with the early stages characterized by the highest consumption. These findings suggest a role for oxidative stress in the early stages of ATTRv.

Published in Antioxidants

ISSN: 2076-3921 (Online)
Publisher: MDPI AG
Country of publisher: Switzerland
LCC subjects: Medicine: Therapeutics. Pharmacology
Website: http://www.mdpi.com/journal/antioxidants

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