Autophagy in desmin-related cardiomyopathy: Thoughts at the halfway point

Alina Maloyan, Jeffrey Robbins

Research output: Contribution to journalShort survey

13 Scopus citations

Abstract

Accumulation of protein aggregates is a hallmark of several neurodegenerative disorders as well as for a number of protein conformation-based diseases, including those affecting muscle, liver and heart. Desminopathy or desminrelated myopathy (DRM) is a skeletal myopathy characterized by bilateral muscle weakness, but is often accompanied by cardiomyopathy as well. DRM can be caused by mutations in desmin, alphaB crystallin, myotilin, Z-band alternatively spliced PDZ-containing protein (ZASP), filamin C (FLNC) or Bcl-2-associated athanogene-3 (BAG3). The common pathological pattern in DRM is accumulation of misfolded proteins, however, clinical manifestations can differ significantly.

Original languageEnglish (US)
Pages (from-to)665-666
Number of pages2
JournalAutophagy
Volume6
Issue number5
DOIs
StatePublished - Jul 1 2010

Keywords

  • Apoptosis
  • Autophagy
  • Cardiomyopathy
  • Cell death
  • Heart failure
  • Protein misfolding

ASJC Scopus subject areas

  • Molecular Biology
  • Cell Biology

Fingerprint Dive into the research topics of 'Autophagy in desmin-related cardiomyopathy: Thoughts at the halfway point'. Together they form a unique fingerprint.

Cite this