Oxidative Stress in Transthyretin-Mediated Amyloidosis: An Exploratory Study.

Autor: Fiore M; CNR-Institute of Biochemistry and Cell Biology, Via Ercole Ramarini 32, 00015 Monterotondo, Italy., Cambieri C; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy., Libonati L; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy., Moret F; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy., D'Andrea E; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy., Di Certo MG; CNR-Institute of Biochemistry and Cell Biology, Via Ercole Ramarini 32, 00015 Monterotondo, Italy., Passananti C; CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy., Gabanella F; CNR-Institute of Biochemistry and Cell Biology, Via Ercole Ramarini 32, 00015 Monterotondo, Italy., Corbi N; CNR-Institute of Molecular Biology and Pathology, Department of Molecular Medicine, Sapienza University of Rome, Viale Regina Elena 291, 00161 Rome, Italy., Garibaldi M; Department of Neuroscience, Mental Health, and Sensory Organs (NESMOS), Sant'Andrea Hospital, Sapienza University, 00189 Rome, Italy., Chimenti C; Department of Clinical, Internal, Anesthesiologist and Cardiovascular Sciences, Sapienza University of Rome, 00185 Rome, Italy., Alfarano M; Department of Clinical, Internal, Anesthesiologist and Cardiovascular Sciences, Sapienza University of Rome, 00185 Rome, Italy., Ferraguti G; Department of Experimental Medicine, Sapienza University of Rome, 00185 Roma, Italy., Francati S; Department of Experimental Medicine, Sapienza University of Rome, 00185 Roma, Italy., Inghilleri M; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy.; IRCCS Neuromed, 86077 Pozzilli, Italy., Ceccanti M; Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy.
Jazyk: angličtina
Zdroj: Antioxidants (Basel, Switzerland) [Antioxidants (Basel)] 2024 Aug 18; Vol. 13 (8). Date of Electronic Publication: 2024 Aug 18.
DOI: 10.3390/antiox13080998
Abstrakt: 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.
Databáze: MEDLINE