Fibrous Proteins: Structures and Mechanisms

Olivier Delalande, Aleksander Czogalla, Jean-François Hubert, Aleksander Sikorski, Elisabeth Le Rumeur, Delalande, Olivier, Czogalla, Aleksander, Hubert, Jean-François, Sikorski, Aleksander, Le Rumeur, Elisabeth

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.