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Current Medicinal Chemistry

Editor-in-Chief

ISSN (Print): 0929-8673
ISSN (Online): 1875-533X

Review Article

Unfolding Cardiac Amyloidosis –From Pathophysiology to Cure

Author(s): Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti and Peter P. Rainer*

Volume 26, Issue 16, 2019

Page: [2865 - 2878] Pages: 14

DOI: 10.2174/0929867325666180104153338

Price: $65

Abstract

Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium causes cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. Thus, the reversal of arrest of adverse cardiac remodeling is the target of current therapies. Here, we provide a condensed overview on the pathophysiology of AL and ATTR cardiac amyloidoses and describe treatments that are currently used or investigated in clinical or preclinical trials. We also briefly discuss acquired amyloid deposition in cardiovascular disease other than AL or ATTR.

Keywords: Amyloidosis, heart failure, pre-amyloid oligomers, fibrils, immunoglobulin light chains, transthyretin, desmin, posttranslational modification.

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