Excitation-contraction coupling alterations in mdx and utrophin/dystrophin double knockout mice: A comparative study

American Journal of Physiology - Cell Physiology

Volumn 298, Issue 5, 2010, Pages

  Capote, Joana ^a   DiFranco, Marino ^a   Vergara, Julio L ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Fluorescence microscopy; Mammalian skeletal muscle; Muscular dystrophy

Indexed keywords

DYSTROPHIN; UTROPHIN;

ACTION POTENTIAL; AFFERENT NERVE GROUP 1; ANIMAL CELL; ANIMAL EXPERIMENT; ARTICLE; CALCIUM TRANSPORT; CONTROLLED STUDY; EXCITATION CONTRACTION COUPLING; KNOCKOUT MOUSE; MORPHOLOGY; MOUSE; MOUSE STRAIN; MUSCLE CELL; MUSCLE EXCITATION; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; SKELETAL MUSCLE; X CHROMOSOME LINKED MUSCULAR DYSTROPHIC MOUSE;

ACTION POTENTIALS; ANIMALS; CALCIUM; DYSTROPHIN; ELECTROPHYSIOLOGY; EXCITATION CONTRACTION COUPLING; MICE; MICE, INBRED MDX; MICE, KNOCKOUT; MUSCLE FIBERS, SKELETAL; MUSCULAR DYSTROPHY, ANIMAL; UTROPHIN;

MAMMALIA; MUS;

EID: 77951446319     PISSN: 03636143     EISSN: 15221563     Source Type: Journal    
DOI: 10.1152/ajpcell.00428.2009     Document Type: Article

References (30)

1. Amalfitano A, Chamberlain JS. The mdx-amplification-resistant mutation system assay, a simple and rapid polymerase chain reaction-based detection of the mdx allele. Muscle Nerve 19: 1549-1553, 1996. (Pubitemid 26412110)
2. Baker PE, Kearney JA, Gong B, Merriam AP, Kuhn DE, Porter JD, and Rafael-Fortney JA. Analysis of gene expression differences between utrophin/dystrophin-deficient vs. mdx skeletal muscles reveals a specific upregulation of slow muscle genes in limb muscles. Neurogenetics 7: 81-91, 2006.
3. Baylor SM, Hollingworth S. Sarcoplasmic reticulum calcium release compared in slow-twitch and fast-twitch fibres of mouse muscle. J Physiol 551: 125-138, 2003. (Pubitemid 37062917)
4. 2+ release displayed by mdx muscle. Biophys J 96: 236a-236a, 2009.
5. Collins CA, Morgan JE. Duchenne's muscular dystrophy: animal models used to investigate pathogenesis and develop therapeutic strategies. Int J Exp Pathol 84: 165-172, 2003.
6. Deconinck AE, Rafael JA, Skinner JA, Brown SC, Potter AC, Metzinger L, Watt DJ, Dickson JG, Tinsley JM, Davies KE. Utrophindystrophin-deficient mice as a model for Duchenne muscular dystrophy. Cell 90: 717-727, 1997. (Pubitemid 27357963)
7. Deconinck N, Rafael JA, Beckers-Bleukx G, Kahn D, Deconinck AE, Davies KE, Gillis JM. Consequences of the combined deficiency in dystrophin and utrophin on the mechanical properties and myosin composition of some limb and respiratory muscles of the mouse. Neuromuscul Disord 8: 362-370, 1998. (Pubitemid 28388934)
8. DiFranco M, Capote J, Vergara JL. Optical imaging and functional characterization of the transverse tubular system of mammalian muscle fibers using the potentiometric indicator di-8-ANEPPS. J Membr Biol 208: 141-153, 2005.
9. DiFranco M, Woods CE, Capote J, Vergara JL. Dystrophic skeletal muscle fibers display alterations at the level of calcium microdomains. Proc Natl Acad Sci USA 105: 14698-14703, 2008.
10. Emery AE. The muscular dystrophies. Lancet 359: 687-695, 2002.
11. Escobar AL, Velez P, Kim AM, Cifuentes F, Fill M, Vergara JL. Kinetic properties of DM-nitrophen and calcium indicators: rapid transient response to flash photolysis. Pflügers Arch 434: 615-631, 1997. (Pubitemid 27355183)
12. Grady RM, Merlie JP, Sanes JR. Subtle neuromuscular defects in utrophin-deficient mice. J Cell Biol 136: 871-882, 1997.
13. Grady RM, Teng H, Nichol MC, Cunningham JC, Wilkinson RS, Sanes JR. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Cell 90: 729-738, 1997. (Pubitemid 27357964)
14. Hennig R, Lomo T. Firing patterns of motor units in normal rats. Nature 314: 164-166, 1985. (Pubitemid 15137124)
15. Hirn C, Shapovalov G, Petermann O, Roulet E, Ruegg Nav1 UT. 4. Deregulation in dystrophic skeletal muscle leads to Na+ overload and enhanced cell death. J Gen Physiol 132: 199-208, 2008.
16. Hollingworth S, Zeiger U, Baylor SM. Comparison of the myoplasmic calcium transient elicited by an action potential in intact fibres of mdx and normal mice. J Physiol 586: 5063-5075, 2008.
17. Keep NH. Structural comparison of actin binding in utrophin and dystrophin. Neurol Sci 21: S929-S937, 2000.
18. Khurana TS, Davies KE. Pharmacological strategies for muscular dystrophy. Nat Rev Drug Discov 2: 379-390, 2003.
19. Nagerl UV, Novo D, Mody I, Vergara JL. Binding kinetics of calbindin-D(28k) determined by flash photolysis of caged Ca(2+). Biophys J 79: 3009-3018, 2000.
20. O'Brien KF, Kunkel LM. Dystrophin and muscular dystrophy: past, present, and future. Mol Genet Metab 74: 75-88, 2001. (Pubitemid 32955149)
21. Peters MF, Adams ME, Froehner SC. Differential association of syntrophin pairs with the dystrophin complex. J Cell Biol 138: 81-93, 1997.
22. Rafael JA, Tinsley JM, Potter AC, Deconinck AE, Davies KE. Skeletal muscle-specific expression of a utrophin transgene rescues utrophindystrophin deficient mice. Nat Genet 19: 79-82, 1998.
23. Rafael JA, Townsend ER, Squire SE, Potter AC, Chamberlain JS, Davies KE. Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure. Hum Mol Genet 9: 1357-1367, 2000.
24. Ribaux P, Bleicher F, Couble ML, Amsellem J, Cohen SA, Berthier C, Blaineau S. Voltage-gated sodium channel (SkM1) content in dystrophindeficient muscle. Pflügers Arch 441: 746-755, 2001. (Pubitemid 32186540)
25. van Deutekom JC, van Ommen GJ. Advances in Duchenne muscular dystrophy gene therapy. Nat Rev Genet 4: 774-783, 2003.
26. Vergara JL, Capote J, DiFranco M. Characterization of the Na and K delayed rectifier conductances of mammalian skeletal muscle fibers' transverse tubular system using the potentiometric indicator di-8-ANEPPS. Biophys J 92: 22a, 2007.
27. Vergara JL, DiFranco M. Sarcoplasmic reticulum calcium content in normal and dystrophic mammalian skeletal muscle fibers. Biophys J 96: 235a-236a, 2009.
28. Watchko JF, O'Day TL, Hoffman EP. Functional characteristics of dystrophic skeletal muscle: insights from animal models. J Appl Physiol 93: 407-417, 2002. (Pubitemid 34810199)
29. Woods CE, Novo D, DiFranco M, Capote J, Vergara JL. Propagation in the transverse tubular system and voltage dependence of calcium release in normal and mdx mouse muscle fibres. J Physiol 568: 867-880, 2005. (Pubitemid 41646027)
30. Woods CE, Novo D, DiFranco M, Vergara JL. The action potential-evoked sarcoplasmic reticulum calcium release is impaired in mdx mouse muscle fibres. J Physiol 557: 59-75, 2004. (Pubitemid 38771637)