Cardiovascular Department, Center for Diagnosis and Treatment of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) and University of Trieste, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart, Via P. Valdoni 7, Trieste 34100, Italy.
Cardiovascular Department, Center for Diagnosis and Treatment of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) and University of Trieste, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart, Via P. Valdoni 7, Trieste 34100, Italy; European Reference Network for Rare Low Prevalence and Complex Diseases of the Heart-ERN GUARD Heart Via P. Valdoni 7 Trieste 34100, Italy.
Heart Fail Clin. 2024 Jul;20(3):249-260. doi: 10.1016/j.hfc.2024.02.001. Epub 2024 Apr 4.
Amyloidosis is a heterogenous group of disorders, caused by the deposition of insoluble fibrils derived from misfolded proteins in the extracellular space of various organs. These proteins have an unstable structure that causes them to misfold, aggregate, and deposit as amyloid fibrils with the pathognomonic histologic property of green birefringence when viewed under cross-polarized light after staining with Congo red. Amyloid fibrils are insoluble and degradation-resistant; resistance to catabolism results in progressive tissue amyloid accumulation. The outcome of this process is organ disfunction independently from the type of deposited protein, however there can be organ that are specifically targeted from certain proteins.
淀粉样变性是一组异质性疾病，由各种器官细胞外空间中源自错误折叠蛋白质的不溶性原纤维沉积引起。这些蛋白质结构不稳定，导致它们错误折叠、聚集，并沉积为淀粉样原纤维，刚果红染色后在偏光显微镜下观察具有特征性的绿色双折射现象。淀粉样纤维是不可溶的且具有抗降解性；抗分解代谢导致组织中淀粉样物质逐渐积累。这个过程的结果是器官功能障碍，与沉积的蛋白质类型无关，但某些蛋白质可以特异性地靶向特定器官。
