Unfolding cardiac amyloidosis –from pathophysiology to cure

Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, Peter P. Rainer

Research output: Contribution to journalReview article

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.

Original languageEnglish (US)
Pages (from-to)2865-2878
Number of pages14
JournalCurrent medicinal chemistry
Volume26
Issue number16
DOIs
StatePublished - Jan 1 2019

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Keywords

  • Amyloidosis
  • Desmin
  • Fibrils
  • Heart failure
  • Immunoglobulin light chains
  • Posttranslational modification
  • Pre-amyloid oligomers
  • Transthyretin

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Medicine
  • Pharmacology
  • Drug Discovery
  • Organic Chemistry

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