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心脏淀粉样变性:分子病理学的最新综述

Cardiac Amyloidosis: State-of-the-Art Review in Molecular Pathology.

作者信息

Salzillo Cecilia, Franco Renato, Ronchi Andrea, Quaranta Andrea, Marzullo Andrea

机构信息

Department of Experimental Medicine, PhD Course in Public Health, University of Campania "Luigi Vanvitelli", 80138 Naples, Italy.

Department of Precision and Regenerative Medicine and Ionian Area, Pathology Unit, University of Bari "Aldo Moro", 70121 Bari, Italy.

出版信息

Curr Issues Mol Biol. 2024 Oct 16;46(10):11519-11536. doi: 10.3390/cimb46100684.

Abstract

Amyloidosis refers to a group of diseases caused by extracellular deposits of misfolded proteins, which alter tissue function and structure, potentially affecting any organ. The term "amyloid" was introduced in the 19th century and later associated with pathological protein deposits. Amyloid fibrils, which are insoluble and resistant to degradation, originate from soluble proteins that undergo misfolding. This process can be triggered by several factors, such as aging, elevated protein concentrations, or pathogenic variants. Amyloid deposits damage organs both by disrupting tissue architecture and through direct cytotoxic effects, leading to conditions such as heart failure. Amyloidosis can be classified into acquired or inherited forms and can be systemic or localized. Diagnosing cardiac amyloidosis is complex and often requires tissue biopsies, which are supported by Congo Red dye staining. In some cases, bisphosphonate bone scans may provide a less invasive diagnostic option. In this state-of-the-art review, we focus on the most common forms of cardiac amyloidosis, from epidemiology to therapy, emphasizing the differences in molecular mechanisms and the importance of pathological diagnosis for appropriate treatment using a multidisciplinary approach.

摘要

淀粉样变性是指由错误折叠的蛋白质在细胞外沉积所引起的一组疾病,这些沉积物会改变组织的功能和结构,可能影响任何器官。“淀粉样蛋白”一词于19世纪被引入,后来与病理性蛋白质沉积物相关联。淀粉样纤维不溶且抗降解,它源自发生错误折叠的可溶性蛋白质。这个过程可由多种因素触发,如衰老、蛋白质浓度升高或致病变体。淀粉样沉积物通过破坏组织结构和产生直接细胞毒性作用来损害器官,导致心力衰竭等病症。淀粉样变性可分为获得性或遗传性形式,可呈全身性或局限性。诊断心脏淀粉样变性很复杂,通常需要组织活检,并通过刚果红染色来辅助诊断。在某些情况下,双膦酸盐骨扫描可能提供一种侵入性较小的诊断选择。在这篇最新综述中,我们聚焦于心脏淀粉样变性最常见的形式,从流行病学到治疗,强调分子机制的差异以及采用多学科方法进行病理诊断对恰当治疗的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174c/11506355/a4088c77a92f/cimb-46-00684-g001.jpg

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