New insights into the molecular basis of desmoplakin and desmin-related cardiomyopathies

Karine Lapouge, Lionel Fantao, Marie France Champliaud, Fabienne Jaunin, Miguel A. Frias, Bertrand Favre, Denise Paulin, Kathleen J. Green, Luca Borradori*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

53 Scopus citations


Desmosomes are intercellular adhesive complexes that anchor the intermediate filament cytoskeleton to the cell membrane in epithelia and cardiac muscle cells. The desmosomal component desmoplakin plays a key role in tethering various intermediate filament networks through its C-terminal plakin repeat domain. To gain better insight into the cytoskeletal organization of cardiomyocytes, we investigated the association of desmoplakin with desmin by cell transfection, yeast two-hybrid, and/or in vitro binding assays. The results indicate that the association of desmoplakin with desmin depends on sequences within the linker region and C-terminal extremity of desmoplakin, where the B and C subdomains contribute to efficient binding; a potentially phosphorylatable serine residue in the C-terminal extremity of desmoplakin affects its association with desmin; the interaction of desmoplakin with non-filamentous desmin requires sequences contained within the desmin C-terminal rod portion and tail domain in yeast, whereas in in vitro binding studies the desmin tail is dispensable for association; and mutations in either the C-terminus of desmoplakin or the desmin tail linked to inherited cardiomyopathy seem to impair desmoplakin-desmin interaction. These studies increase our understanding of desmoplakin-intermediate filament interactions, which are important for maintenance of cytoarchitecture in cardiomyocytes, and give new insights into the molecular basis of desmoplakin- and desmin-related human diseases.

Original languageEnglish (US)
Pages (from-to)4974-4985
Number of pages12
JournalJournal of cell science
Issue number23
StatePublished - Dec 1 2006


  • Cardiomyocyte
  • Cardiomyopathy
  • Desmin
  • Desmoplakin
  • Intermediate filaments
  • Vimentin

ASJC Scopus subject areas

  • Cell Biology


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