title: Dystrophin and Spectrin, Two Highly Dissimilar Sisters of the Same Family.
abstract: Dystrophin and Spectrin are two proteins essential for the organization of the cytoskeleton and for the stabilization of membrane cells. The comparison of these two sister proteins, and with the dystrophin homologue utrophin, enables us to emphasise that, despite a similar topology with common subdomains and a common structural basis of a three-helix coiled-coil, they show a large range of dissimilarities in terms of genetics, cell expression and higher level structural organisation. Interactions with cellular partners, including proteins and membrane phospholipids, also show both strikingly similar and very different behaviours. The differences between dystrophin and spectrin are also illustrated by the large variety of pathological anomalies emerging from the dysfunction or the absence of these proteins, showing that they are keystones in their function of providing a scaffold that sustains cell structure.
authors.affiliation: Institut de Génétique et Développement de Rennes, UMR CNRS 6290, Université de Rennes 1, Rennes, France. olivier.delalande@univ-rennes1.fr.
authors.forename: Olivier
authors.initials: O
authors.lastname: Delalande
authors.affiliation: Biotechnology Faculty, Department of Cytobiochemistry, University of Wrocław, ul. joliot-Curie 14a, 50-383, Wroclaw, Poland.
authors.forename: Aleksander
authors.initials: A
authors.lastname: Czogalla
authors.affiliation: Institut de Génétique et Développement de Rennes, UMR CNRS 6290, Université de Rennes 1, Rennes, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Hubert
authors.affiliation: Biotechnology Faculty, Department of Cytobiochemistry, University of Wrocław, ul. joliot-Curie 14a, 50-383, Wroclaw, Poland.
authors.forename: Aleksander
authors.initials: A
authors.lastname: Sikorski
authors.affiliation: Institut de Génétique et Développement de Rennes, UMR CNRS 6290, Université de Rennes 1, Rennes, France.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Le Rumeur
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D003599:Cytoskeleton / Q000737:chemistry* / Q000648:ultrastructure; D016189:Dystrophin / Q000737:chemistry* / Q000648:ultrastructure; D006801:Humans; D011487:Protein Conformation; D013049:Spectrin / Q000737:chemistry* / Q000648:ultrastructure
chemical_list: D016189:Dystrophin; D013049:Spectrin
keywords: Coiled-coil fold; Dystrophin; Genetic disease; Membrane binding; Scaffolding protein; Spectrin