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Review
. 2024 Jul;20(3):249-260.
doi: 10.1016/j.hfc.2024.02.001. Epub 2024 Apr 4.

Amyloidosis and Amyloidogenesis: One Name, Many Diseases

Marco Pozzan  1 , Carla Indennidate  1 , Guerino Giuseppe Varrà  1 , Gianfranco Sinagra  1 , Marco Merlo  2 , Linda Pagura  3
Affiliations
Review

Amyloidosis and Amyloidogenesis: One Name, Many Diseases

Marco Pozzan et al. Heart Fail Clin. 2024 Jul.

Abstract

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.

Keywords: ATTR; Amyloidogenesis; Amyloidosis; Cardiac amyloidosis; Insoluble fibrils.

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Conflict of interest statement

Disclosure No conflicts of interest to declare for any author in relation to the submitted work. Outside the submitted work: G. Sinagra received personal fees for occasional educational activities from Biotronik, Boston Scientific, Astra Zeneca and Novartis; M. Merlo received congress fees from Novartis, Vifor Pharma, Astra Zeneca and Novo Nordisk and research grant and congress fees from Pfizer, without any control on intellectual contents.