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
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.
