转甲状腺素蛋白介导的淀粉样变性中的氧化应激：一项探索性研究。
Oxidative Stress in Transthyretin-Mediated Amyloidosis: An Exploratory Study.
作者信息
Fiore Marco, Cambieri Chiara, Libonati Laura, Moret Federica, D'Andrea Edoardo, Di Certo Maria Grazia, Passananti Claudio, Gabanella Francesca, Corbi Nicoletta, Garibaldi Matteo, Chimenti Cristina, Alfarano Maria, Ferraguti Giampiero, Francati Silvia, Inghilleri Maurizio, Ceccanti Marco
机构信息
CNR-Institute of Biochemistry and Cell Biology, Via Ercole Ramarini 32, 00015 Monterotondo, Italy.
Center for Rare Neuromuscular Diseases, Department of Human Neuroscience, Sapienza University of Rome, Viale dell'Università 30, 00185 Rome, Italy.
出版信息
Antioxidants (Basel). 2024 Aug 18;13(8):998. doi: 10.3390/antiox13080998.
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.
转甲状腺素蛋白介导的淀粉样变性（ATTR）是一种全身性疾病，蛋白质在许多组织中沉淀，主要是周围神经和心脏。已知有遗传型（ATTRv，“v”代表变异型）和野生型（ATTRwt）两种形式。除了空间位阻外，沉淀的转甲状腺素蛋白似乎还有毒性作用。在这项针对男性进行的研究中，我们招募了15名ATTRv患者、7名ATTRv无症状携带者、14名ATTRwt患者以及10名年轻和13名老年健康对照者，使用FORD（游离氧自由基防御）和FORT（游离氧自由基测试）分析来评估氧化应激。与ATTRwt和ATTRv无症状携带者相比，ATTRv患者的FORD降低。FORD独立预测疾病阶段，早期阶段的特点是消耗量最高。这些发现表明氧化应激在ATTRv的早期阶段起作用。
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