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

Alina Maloyan, Jeffrey Robbins

Research output: Contribution to journalArticle

12 Citations (Scopus)

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
Externally publishedYes

Fingerprint

Desmin
Autophagy
Muscular Diseases
Cardiomyopathies
Filamins
Protein Conformation
Crystallins
Muscle Weakness
Protein C
Neurodegenerative Diseases
Myocardium
Mutation
Liver
Proteins

Keywords

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

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology

Cite this

Autophagy in desmin-related cardiomyopathy : Thoughts at the halfway point. / Maloyan, Alina; Robbins, Jeffrey.

In: Autophagy, Vol. 6, No. 5, 01.07.2010, p. 665-666.

Research output: Contribution to journalArticle

Maloyan, Alina ; Robbins, Jeffrey. / Autophagy in desmin-related cardiomyopathy : Thoughts at the halfway point. In: Autophagy. 2010 ; Vol. 6, No. 5. pp. 665-666.
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