Ischemia reperfusion injury provokes adverse left ventricular remodeling in dysferlin-deficient hearts through a pathway that involves TIRAP dependent signaling

Sci Rep. 2020 Aug 24;10(1):14129. doi: 10.1038/s41598-020-71079-7.

Abstract

Cardiac myocytes have multiple cell autonomous mechanisms that facilitate stabilization and repair of damaged sarcolemmal membranes following myocardial injury. Dysferlin is a protein which facilitates membrane repair by promoting membrane resealing. Although prior studies have shown that dysferlin-deficient (Dysf-/-) mouse hearts have an impaired recovery from acute ischemia/reperfusion (I/R) injury ex vivo, the role of dysferlin in mediating the recovery from myocardial injury in vivo is unknown. Here we show that Dysf-/- mice develop adverse LV remodeling following I/R injury secondary to the collateral damage from sustained myocardial inflammation within the infarct zone. Backcrossing Dysf-/- mice with mice lacking signaling through the Toll-Interleukin 1 Receptor Domain-Containing Adaptor Protein (Tirap-/-), attenuated inflammation and abrogated adverse LV remodeling following I/R injury. Subsequent studies using Poloxamer 188 (P188), a membrane resealing reagent, demonstrated that P188 did not attenuate inflammation nor prevent adverse LV remodeling in Dysf-/- mice following I/R injury. Viewed together these studies reveal a previously unappreciated role for the importance of membrane sealing and the resolution of inflammation following myocardial injury.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Cardiotonic Agents / pharmacology
  • Dysferlin / deficiency
  • Dysferlin / genetics*
  • Inflammation / pathology
  • Membrane Glycoproteins / genetics
  • Membrane Glycoproteins / metabolism*
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Myocardial Ischemia / pathology*
  • Myocardium / pathology
  • Phospholipids / metabolism
  • Poloxamer / pharmacology
  • Receptors, Interleukin-1 / genetics
  • Receptors, Interleukin-1 / metabolism*
  • Reperfusion Injury / pathology*
  • Sarcolemma / physiology
  • Signal Transduction
  • Surface-Active Agents / pharmacology
  • Ventricular Remodeling / physiology*

Substances

  • Cardiotonic Agents
  • Dysf protein, mouse
  • Dysferlin
  • Membrane Glycoproteins
  • Phospholipids
  • Receptors, Interleukin-1
  • Surface-Active Agents
  • TIRAP protein, mouse
  • Poloxamer