血清和组织轻链作为AL淀粉样变性的疾病生物标志物和治疗靶点

Vessel plus Pub Date : 2022-01-01 DOI:10.20517/2574-1209.2022.06

L. Camerini, A. Aimo, A. Pucci, V. Castiglione, V. Musetti, S. Masotti, L. Caponi, G. Vergaro, C. Passino, A. Clerico, M. Franzini, M. Emdin

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引用次数: 0

摘要

淀粉样蛋白轻链(AL)淀粉样变性是最常见的系统性淀粉样变性，是一种多器官疾病，主要影响心脏和肾脏。AL淀粉样变性是一种蛋白质错误折叠疾病，其特征是肿瘤浆细胞产生单克隆轻链(LCs)的组织沉积。循环游离LC (FLC)的测量是AL淀粉样变性诊断、风险分层和管理的重要工具，可以通过基于抗体的方法或质谱法进行。此外，正确识别组织中的LC沉积物对于诊断AL淀粉样变性至关重要。与基于抗体的技术一起，依靠质谱的方法现在是可用的。

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Serum and tissue light-chains as disease biomarkers and targets for treatment in AL amyloidosis

Amyloid light-chain (AL) amyloidosis is the most common type of systemic amyloidosis and is a multi-organ disease affecting mostly the heart and kidneys. AL amyloidosis is a protein misfolding disorder characterized by the tissue deposition of monoclonal light chains (LCs) produced by neoplastic plasma cells. Measurement of circulating free LC (FLC) is an important tool for diagnosis, risk stratification, and management of AL amyloidosis and can be performed through antibody-based methods or mass spectrometry. Furthermore, correct identification of LC deposits in tissues is essential to diagnose AL amyloidosis. Together with antibody-based techniques, methods relying on mass spectroscopy are now available.

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来源期刊

Vessel plus

CiteScore

1.80

自引率

0.00%

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