The Journal of General Physiology
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Published online Jan 30 2006. doi:10.1085/jgp.200509456
The Rockefeller University Press, 0022-1295 $8.00
JGP, Volume 127, Number 2, 171-182
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ARTICLE

 Mini-dystrophin Expression Down-regulates Overactivation of G Protein–mediated IP3 Signaling Pathway in Dystrophin-deficient Muscle Cells

Haouaria Balghi, Stéphane Sebille, Bruno Constantin, Sylvie Patri, Vincent Thoreau, Ludivine Mondin, Elise Mok, Alain Kitzis, Guy Raymond, and Christian Cognard

Institut de Physiologie et Biologie Cellulaires, CNRS UMR 6187, Université de Poitiers, 86022 Poitiers, France

Correspondence to Stéphane Sebille: stephane.sebille{at}univ-poitiers.fr

We present here evidence for the enhancement of an inositol 1,4,5-trisphosphate (IP3) mediated calcium signaling pathway in myotubes from dystrophin-deficient cell lines (SolC1(–)) as compared to a cell line from the same origin but transfected with mini-dystrophin (SolD(+)). With confocal microscopy, we demonstrated that calcium rise, induced by the perifusion of a solution containing a high potassium concentration, was higher in SolC1(–) than in SolD(+) myotubes. The analysis of amplitude and kinetics of the calcium increase in SolC1(–) and in SolD(+) myotubes during the exposure with SR Ca2+ channel inhibitors (ryanodine and 2-APB) suggested the presence of two mechanisms of SR calcium release: (1) a fast SR calcium release that depended on ryanodine receptors and (2) a slow SR calcium release mediated by IP3 receptors. Detection analyses of mRNAs (reverse transcriptase [RT]-PCR) and proteins (Western blot and immunolocalization) demonstrated the presence of the three known isoforms of IP3 receptors in both SolC1(–) and SolD(+) myotubes. Furthermore, analysis of the kinetics of the rise in calcium revealed that the slow IP3-dependent release may be increased in the SolC1(–) as compared to the SolD(+), suggesting an inhibitory effect of mini-dystrophin in this signaling pathway. Upon incubation with pertussis toxin (PTX), an inhibitory effect similar to that of the IP3R inhibitor (2-APB) was observed on K+-evoked calcium release. This result suggests the involvement of a Gi protein upstream of the IP3 pathway in these stimulation conditions. A hypothetical model is depicted in which both Gi protein and IP3 production could be involved in K+-evoked calcium release as well as a possible interaction with mini-dystrophin. Our findings demonstrate the existence of a potential relationship between mini-dystrophin and SR calcium release as well as a regulatory role of mini-dystrophin on intracellular signaling.

Abbreviations used in this paper: BMD, Becker muscular dystrophy; CICR, calcium-induced calcium release; DAP, dystrophin-associated protein; DHPR, dihydropyridine receptor; DMD, Duchenne muscular dystrophy; HD, half decay; HRP, horseradish peroxidase; IP3, inositol 1,4,5-trisphosphate; IP3R, IP3 receptor; IS, increase slope; PTX, pertussis toxin; ROI, region of interest; RT, reverse transcriptase; RYR, ryanodine receptor; TTP, time to peak.


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