New insights in the regulation of calcium transfers by muscle dystrophin-based cytoskeleton: implications in DMD - Laboratoire Signalisation et Transports Ioniques Membranaires Accéder directement au contenu
Article Dans Une Revue Journal of Muscle Research and Cell Motility Année : 2006

New insights in the regulation of calcium transfers by muscle dystrophin-based cytoskeleton: implications in DMD

Résumé

Calcium mishandling in Duchenne muscular dystrophy (DMD) suggested that dystrophin, a membrane-associated cytoskeleton protein, may regulate calcium-signalling cascades such as calcium entries. Calcium overload in human DMD myotubes is dependent on their contractile activity suggesting the involvement of channels being activated during contraction and/or calcium release. Forced expression of mini-dystrophin in dystrophin-deficient myotubes, reactivates appropriate sarcolemmal expression of dystrophin-associated proteins and restores normal calcium handling in the cytosol. Furthermore, the recombinant mini-dystrophin reduced the store-operated calcium influx across the sarcolemma, and the mitochondrial calcium uptake during this influx. A slow component of calcium release dependent on IP3R, as well as the production of IP3, were also reduced to normal levels by expression of mini-dystrophin. Our studies provide a new model for the convergent regulation of transmembrane calcium influx and IP3-dependent calcium release by the dystrophin-based cytoskeleton (DBC). We also suggest molecular association of such channels with DBC which may provide the scaffold for assembling a multiprotein-signalling complex that modulates the channel activity. This suggests that the loss of this molecular association could participate in the alteration of calcium homeostasis observed in DMD muscle cells.
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Dates et versions

hal-02880158 , version 1 (18-11-2022)

Identifiants

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Bruno Constantin, Stéphane Sebille, Christian Cognard. New insights in the regulation of calcium transfers by muscle dystrophin-based cytoskeleton: implications in DMD. Journal of Muscle Research and Cell Motility, 2006, 27 (5-7), pp.375-386. ⟨10.1007/s10974-006-9085-2⟩. ⟨hal-02880158⟩
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