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Amyloidosis and Amyloidogenesis: One Name, Many Diseases

Pozzan, Marco
•
Indennidate, Carla
•
Varrà, Guerino Giuseppe
altro
Pagura, Linda
2024
  • journal article

Periodico
HEART FAILURE CLINICS
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.
DOI
10.1016/j.hfc.2024.02.001
WOS
WOS:001257108600001
Archivio
https://hdl.handle.net/11368/3077318
info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85189428517
https://www.sciencedirect.com/science/article/abs/pii/S1551713624000151?via=ihub
Diritti
closed access
license:copyright editore
license uri:iris.pri02
FVG url
https://arts.units.it/request-item?handle=11368/3077318
Soggetti
  • ATTR

  • Amyloidogenesi

  • Amyloidosi

  • Cardiac amyloidosi

  • Insoluble fibrils

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