| Literature DB >> 29303069 |
Klemens Ablasser1, Nicolas Verheyen1, Theresa Glantschnig1, Giulio Agnetti2,3, Peter P Rainer1.
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. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.Entities:
Keywords: Amyloidosis; desmin; fibrils; heart failure; immunoglobulin light chains; posttranslational modification; pre-amyloid oligomers; transthyretin.
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Year: 2019 PMID: 29303069 DOI: 10.2174/0929867325666180104153338
Source DB: PubMed Journal: Curr Med Chem ISSN: 0929-8673 Impact factor: 4.530