Unfolding Cardiac Amyloidosis –From Pathophysiology to Cure

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

Journal Name: Current Medicinal Chemistry


Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause 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. The reversal or arrest of adverse cardiac remodeling is the target of current therapies, as cardiac-related mortality worsens prognosis in patients where the underlying systemic amyloidosis was successfully treated. 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 touch on 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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(E-pub Ahead of Print)
DOI: 10.2174/0929867325666180104153338
Price: $95

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