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

Détails

ID Serval
serval:BIB_7E51FDCB2766
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
New insights into the molecular basis of desmoplakin- and desmin-related cardiomyopathies.
Périodique
Journal of Cell Science
Auteur(s)
Lapouge K., Fontao L., Champliaud M.F., Jaunin F., Frias M.A., Favre B., Paulin D., Green K.J., Borradori L.
ISSN
0021-9533 (Print)
ISSN-L
0021-9533
Statut éditorial
Publié
Date de publication
2006
Volume
119
Numéro
Pt 23
Pages
4974-4985
Langue
anglais
Résumé
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 desmoplakindesmin 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.
Mots-clé
Animals, Animals, Newborn, Cardiomyopathies/genetics, Cells, Cultured, Desmin/genetics, Desmin/metabolism, Desmoplakins/genetics, Desmoplakins/metabolism, Humans, Mutant Proteins/metabolism, Mutation, Myocytes, Cardiac/metabolism, Protein Binding, Protein Structure, Tertiary, Rats, Rats, Wistar, Sequence Analysis, Protein, Tissue Distribution, Transfection, Two-Hybrid System Techniques
Pubmed
Web of science
Création de la notice
25/01/2008 17:32
Dernière modification de la notice
03/03/2018 18:41
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