Proteasome inhibitor (MG132) rescues Nav1.5 protein content and the cardiac sodium current in dystrophin-deficient mdx5cv mice

Frontiers in Physiology

Volumn 4 MAR, Issue , 2013, Pages

  Rougier, Jean Sébastien ^a   Gavillet, Bruno ^a   Abrie, Hugues ^a  

^a UNIVERSITY OF BERN   (Switzerland)

Author keywords

Dystrophin; Electrophysiology; Mg132; Proteasome; Proteasome inhibitors; Sodium channels

Indexed keywords

BENZYLOXYCARBONYLLEUCYLLEUCYLLEUCINAL; DYSTROPHIN; MESSENGER RNA; PROTEASOME; SODIUM; SODIUM CHANNEL NAV1.5; UBIQUITIN PROTEIN LIGASE NEDD4; VOLTAGE GATED SODIUM CHANNEL; VOLTAGE GATED SODIUM CHANNEL BETA 1 SUBUNIT;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; CELL MEMBRANE; CONTROLLED STUDY; HEART CONDUCTION; HEART MUSCLE; HEART MUSCLE CELL; HEART MUSCLE EXCITABILITY; MALE; MOUSE; NONHUMAN; OSMOTIC MINIPUMP; PROTEIN CONTENT; PROTEIN DEGRADATION; PROTEIN EXPRESSION; SKELETAL MUSCLE; SODIUM CURRENT; WILD TYPE;

EID: 84879495401     PISSN: None     EISSN: 1664042X     Source Type: Journal    
DOI: 10.3389/fphys.2013.00051     Document Type: Article

References (29)

1. v1.5 by utrophin in dystrophin-deficient mice. Cardiovasc. Res. 89, 320-328.
2. Antzelevitch, C. (2001). The Brugada syndrome: ionic basis and arrhythmia mechanisms. J. Cardiovasc. Electrophysiol. 12, 268-272.
3. Assereto, S., Stringara, S., Sotgia, F., Bonuccelli, G., Broccolini, A., Pedemonte, M., et al. (2006). Pharmacological rescue of the dystrophin-glycoprotein complex in Duchenne and Becker skeletal muscle explants by proteasome inhibitor treatment. Am. J. Physiol. Cell Physiol. 290, C577-C582.
4. Barnabei, M. S., and Metzger, J. M. (2012). Ex vivo stretch reveals altered mechanical properties of isolated dystrophin-deficient hearts. PLoS ONE 7:e32880. doi: 10.1371/journal.pone.0032880
5. Bonuccelli, G., Sotgia, F., Schubert, W., Park, D. S., Frank, P. G., Woodman, S. E., et al. (2003). Proteasome inhibitor (MG-132) treatment of mdx mice rescues the expression and membrane localization of dystrophin and dystrophin-associated proteins 1. Am. J. Pathol. 163, 1663-1675.
6. Danko, I., Chapman, V., and Wolff, J. A. (1992). The frequency of revertants in mdx mouse genetic models for Duchenne muscular dystrophy. Pediatr. Res. 32, 128-131.
7. v1.5 is regulated by a multiprotein complex composed of syntrophins and dystrophin. Circ. Res. 99, 407-414.
8. Gee, S. H., Madhavan, R., Levinson, S. R., Caldwell, J. H., Sealock, R., and Froehner, S. C. (1998). Interaction of muscle and brain sodium channels with multiple members of the syntrophin family of dystrophin-associated proteins. J. Neurosci. 18, 128-137.
9. Haslett, J. N., Sanoudou, D., Kho, A. T., Bennett, R. R., Greenberg, S. A., Kohane, I. S., et al. (2002). Gene expression comparison of biopsies from Duchenne muscular dystrophy (DMD) and normal skeletal muscle. Proc. Natl. Acad. Sci. U.S.A. 99, 15000-15005.
10. Hesse, M., Kondo, C. S., Clark, R. B., Su, L., Allen, F. L., Geary-Joo, C. T., et al. (2007). Dilated cardiomyopathy is associated with reduced expression of the cardiac sodium channel Scn5a. Cardiovasc. Res. 75, 498-509.
11. Im, W. B., Phelps, S. F., Copen, E. H., Adams, E. G., Slightom, J. L., and Chamberlain, J. S. (1996). Differential expression of dystrophin isoforms in strains of mdx mice with different mutations. Hum. Mol. Genet. 5, 1149-1153.
12. Lee, D. H., and Goldberg, A. L. (1998). Proteasome inhibitors: valuable new tools for cell biologists. Trends in Cell Biol. 8, 397-403.
13. Longva, K. E., Blystad, F. D., Stang, E., Larsen, A. M., Johannessen, L. E., and Madshus, I. H. (2002). Ubiquitination and proteasomal activity is required for transport of the EGF receptor to inner membranes of multivesicular bodies. J. Cell Biol. 156, 843-854.
14. Mann, S. A., Castro, M. L., Ohanian, M., Guo, G., Zodgekar, P., Sheu, A., et al. (2012). R222Q SCN5A mutation is associated with reversible ventricular ectopy and dilated cardiomyopathy. J. Am. Coll. Cardiol. 60, 1566-1573.
15. Mcnair, W. P., Ku, L., Taylor, M. R. G., Fain, P. R., Dao, D., Wolfel, E., et al. (2004). SCN5A mutation associated with dilated cardiomyopathy, conduction disorder, and arrhythmia. Circulation 110, 2163-2167.
16. Mohler, P. J., and Bennett, V. (2005). Ankyrin-based cardiac arrhythmias: a new class of channelopathies due to loss of cellular targeting. Curr. Opin. Cardiol. 20, 189-193.
17. Moric, E., Herbert, E., Trusz-Gluza, M., Filipecki, A., Mazurek, U., and Wilczok, T. (2003). The implications of genetic mutations in the sodium channel gene (SCN5A). Europace 5, 325-334.
18. Nerbonne, J. M., and Kass, R. S. (2005). Molecular physiology of cardiac repolarization. Physiol. Rev. 85, 1205-1253.
19. v1.5 in cardiomyocytes. Circ. Res. 108, 294-304.
20. Rock, K. L., Gramm, C., Rothstein, L., Clark, K., Stein, R., Dick, L., et al. (1994). Inhibitors of the proteasome block the degradation of most cell proteins and the generation of peptides presented on MHC class I molecules. Cell 78, 761-771.
21. Rougier, J. S., van Bemmelen, M. X., Bruce, M. C., Jespersen, T., Gavillet, B., Apotheloz, F., et al. (2005). Molecular determinants of voltage-gated sodium channel regulation by the Nedd4/Nedd4-like proteins. Am. J. Physiol. Cell Physiol. 288, C692-C701.
22. Salghetti, S. E., Caudy, A. A., Chenoweth, J. G., and Tansey, W. P. (2001). Regulation of transcriptional activation domain function by ubiquitin. Science 293, 1651-1653.
23. Tan, H. L., Bezzina, C. R., Smits, J. P. P., Verkerk, A. O., and Wilde, A. A. (2003). Genetic control of sodium channel function. Cardiovasc. Res. 57, 961-973.
24. Towbin, J. A., Hejtmancik, J. F., Brink, P., Gelb, B., Zhu, X. M., Chamberlain, J. S., et al. (1993). X-linked dilated cardiomyopathy. Molecular genetic evidence of linkage to the Duchenne muscular dystrophy (dystrophin) gene at the Xp21 locus. Circulation 87, 1854-1865.
25. v1.5 is regulated by Nedd4-2 mediated ubiquitination. Circ. Res. 95, 284-291.
26. Wang, Q., Shen, J., Li, Z., Timothy, K., Vincent, G. M., Priori, S. G., et al. (1995a). Cardiac sodium channel mutations in patients with long QT syndrome, an inherited cardiac arrhythmia. Hum. Mol. Genet. 4, 1603-1607.
27. Wang, Q., Shen, J., Splawski, I., Atkinson, D., Li, Z., Robinson, J. L., et al. (1995b). SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell 80, 805-811.
28. Wilde, A. A., Antzelevitch, C., Borggrefe, M., Brugada, J., Brugada, R., Brugada, P., et al. (2002). Proposed diagnostic criteria for the Brugada syndrome: consensus report. Circulation 106, 2514-2519.
29. Yu, E. J., Ko, S. H., Lenkowski, P. W., Pance, A., Patel, M. K., and Jackson, A. P. (2005). Distinct domains of the sodium channel beta3 subunit modulate channel gating kinetics and sub-cellular location. Biochem. J. 392(Pt 3), 519-526.