MLP and CARP are linked to chronic PKCα signalling in dilated cardiomyopathy

Nat Commun. 2016 Jun 29:7:12120. doi: 10.1038/ncomms12120.

Abstract

MLP (muscle LIM protein)-deficient mice count among the first mouse models for dilated cardiomyopathy (DCM), yet the exact role of MLP in cardiac signalling processes is still enigmatic. Elevated PKCα signalling activity is known to be an important contributor to heart failure. Here we show that MLP directly inhibits the activity of PKCα. In end-stage DCM, PKCα is concentrated at the intercalated disc of cardiomyocytes, where it is sequestered by the adaptor protein CARP in a multiprotein complex together with PLCβ1. In mice deficient for both MLP and CARP the chronic PKCα signalling chain at the intercalated disc is broken and they remain healthy. Our results suggest that the main role of MLP in heart lies in the direct inhibition of PKCα and that chronic uninhibited PKCα activity at the intercalated disc in the absence of functional MLP leads to heart failure.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • COS Cells
  • Cardiomyopathy, Dilated / metabolism*
  • Cell Line
  • Chlorocebus aethiops
  • Escherichia coli
  • Gene Expression Regulation
  • Heart Failure / etiology
  • Humans
  • LIM Domain Proteins / genetics
  • LIM Domain Proteins / metabolism*
  • Male
  • Mice
  • Muscle Proteins / genetics
  • Muscle Proteins / metabolism*
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism*
  • Protein Kinase C-alpha / genetics
  • Protein Kinase C-alpha / metabolism*
  • Repressor Proteins / genetics
  • Repressor Proteins / metabolism*
  • Signal Transduction

Substances

  • ANKRD1 protein, human
  • Ankrd1 protein, mouse
  • LIM Domain Proteins
  • Muscle Proteins
  • Nuclear Proteins
  • Repressor Proteins
  • cysteine and glycine-rich protein 3
  • Protein Kinase C-alpha