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Unfolding Cardiac Amyloidosis –From Pathophysiology to Cure

[ Vol. 26 , Issue. 16 ]

Author(s):

Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti and Peter P. Rainer*   Pages 2865 - 2878 ( 14 )

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.

Affiliation:

Division of Cardiology, Medical University of Graz, Graz, Division of Cardiology, Medical University of Graz, Graz, Division of Cardiology, Medical University of Graz, Graz, Division of Cardiology, Johns Hopkins School of Medicine, Baltimore, MD, Division of Cardiology, Medical University of Graz, Graz



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