Abnormal Na channel gating in murine cardiac myocytes deficient in myotonic dystrophy protein kinase

Physiological Genomics

Volumn 12, Issue , 2003, Pages 147-157

  Lee, Hwa C ^d   Patel, Manoj K ^a   Mistry, Dilawaar J ^a   Wang, Qingcai ^b   Reddy, Sita ^b   Moorman, J Randall ^a   Mounsey, J Paul ^a,c  

^a UNIVERSITY OF VIRGINIA HEALTH SYSTEM   (United States)

^b UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

^c VETERANS AFFAIRS MEDICAL CENTER   (United States)

^d NONE

Author keywords

Action potential; Cardiac myocytes; Protein kinase; Sodium ion channel

Indexed keywords

ION CHANNEL; MESSENGER RNA; MYOTONIC DYSTROPHY PROTEIN KINASE; PROTEIN KINASE; PROTEIN SERINE THREONINE KINASE; SODIUM CHANNEL; SODIUM ION; UNCLASSIFIED DRUG;

ACTION POTENTIAL; ANIMAL CELL; ARTICLE; ENZYME DEFICIENCY; HEART ELECTROPHYSIOLOGY; HEART MUSCLE CELL; HEART MUSCLE POTENTIAL; HEART VENTRICLE WALL; MUSCULAR DYSTROPHY; MYOTONIC DYSTROPHY; NONHUMAN; PATCH CLAMP; PRIORITY JOURNAL; ANIMAL; CELL MEMBRANE POTENTIAL; CHANNEL GATING; CHEMISTRY; CONGENIC STRAIN; ENZYMOLOGY; GENETICS; METABOLISM; MOUSE; MOUSE MUTANT; MOUSE STRAIN; PHYSIOLOGY;

ANIMALIA; MURINAE;

ACTION POTENTIALS; ANIMALS; ANIMALS, CONGENIC; ION CHANNEL GATING; MEMBRANE POTENTIALS; MICE; MICE, INBRED STRAINS; MICE, KNOCKOUT; MYOCYTES, CARDIAC; MYOTONIC DYSTROPHY; PATCH-CLAMP TECHNIQUES; PROTEIN-SERINE-THREONINE KINASES; SODIUM CHANNELS;

EID: 0141818030     PISSN: 15312267     EISSN: None     Source Type: Journal    
DOI: 10.1152/physiolgenomics.00095.2002     Document Type: Article

References (39)

1. Aslanidis C, Jansen G, Amemiya C, Shutler G, Mahadevan M, Tsilfidis C, Chen C, Alleman J, Wormskamp NG, Vooijs M, Buxton J, Johnson K, Smeets HJM, Lennon GC, Carrano AV, Korneluk RG, Wieringa B, and de Jong PJ. Cloning of the essential myotonic dystrophy region and mapping of the putative defect. Nature 355: 548-551, 1992.
2. + channel by protein kinase C. Proc Natl Acad Sci USA 92: 12003-12007, 1995.
3. Bennett PB, Yazawa K, Makita N, and George ALJ. Molecular mechanism for an inherited cardiac arrhythmia. Nature 376: 683-685, 1995.
4. + channels. J Membr Biol 130: 183-190, 1992.
5. Berul CI, Maguire CT, Aronovitz MJ, Greenwood J, Miller C, Gehrmann J, Housman D, Mendelsohn ME, and Reddy S. DMPK dosage alterations result in atrioventricular conduction abnormalities in a mouse myotonic dystrophy model. J Clin Invest 103: R1-R7, 1999.
6. Bhagwati S, Ghatpande A, and Leung B. Normal levels of DM RNA and myotonin protein kinase in skeletal muscle from adult myotonic dystrophy (DM) patients. Biochim Biophys Acta 1317: 155-157, 1996.
7. Brook JD, McCurrach ME, Harley HG, Buckler AJ, Church D, Aburatani H, Hunter K, Stanton VP, Thirion JP, Hudson T, Sohn R, Zemelman B, Snell RG, Rundle SA, Crow S, Davies J, Shelbourne P, Buxton J, Jones C, Juvonen V, Johnson K, Harper PS, Shaw DJ, and Housman DE. Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3′ end of a transcript encoding a protein kinase family member. Cell 68: 799-808, 1992.
8. Carango P, Noble JE, Marks HG, and Funanage VL. Absence of myotonic dystrophy protein kinase (DMPK) mRNA as a result of a triplet repeat expansion in myotonic dystrophy. Genomics 18: 340-348, 1993.
9. Chahine M and George AL. Myotonic dystrophy kinase modulates skeletal muscle but not cardiac voltage-gated sodium channels. FEBS Lett 412: 621-624, 1997.
10. - conductance in resealed muscle fibre segments from patients with myotonic dystrophy. J Physiol 425: 391-405, 1990.
11. Frohnwieser B, Weigl L, and Schreibmayer W. Modulation of cardiac sodium channel isoform by cyclic AMP dependent protein kinase does not depend on phosphorylation of serine 1504 in the cytosolic loop interconnecting transmembrane domains III and IV. Pflügers Arch 430: 751-753, 1995.
12. Fu YH, Friedman DL, Richards S, Pearlman JA, Gibbs RA, Pizzuti A, Ashizawa T, Perryman MB, Scarlato G, Fenwick RG, and Caskey CT. Decreased expression of myotonin-protein kinase messenger RNA and protein in adult form of myotonic dystrophy. Science 260: 235-238, 1993.
13. Fu YH, Pizzuti A, Fenwick RJ, King J, Rajnarayan S, Dunne PW, Dubel J, Nasser GA, Ashizawa T, de Jong P, Wieringa B, Korneluk RG, Perryman MB, Epstein HF, and Caskey CT. An unstable triplet repeat in a gene related to myotonic muscular dystrophy. Science 255: 1256-1258, 1992.
14. Grant AO and Starmer CF. Mechanisms of closure of cardiac sodium channels in rabbit ventricular myocytes: single-channel analysis. Circ Res 60: 897-913, 1987.
15. Jan LY and Jan YN. A superfamily of ion channels. Nature 345: 672-672, 1990.
16. Jansen G, Mahadevan M, Amemiya C, Wormskamp N, Segers B, Hendriks W, O'Hoy K, Baird S, Sabourin L, and Lennon G. Characterization of the myotonic dystrophy region predicts multiple protein isoform-encoding mRNAs. Nat Genet 1: 261-266, 1992.
17. Kunze DL, Lacerda AE, Wilson DL, and Brown AM. Cardiac Na currents and the inactivating, reopening, and waiting properties of single cardiac Na channels. J Gen Physiol 86: 691-719, 1985.
18. Magistretti J, Mantegazza M, Guatteo E, and Wanke E. Action potentials recorded with patch clamp amplifiers. Are they genuine? Trends Neurosci 19: 530-534, 1996.
19. Mahadevan M, Tsilfidis C, Sabourin L, Shutler G, Amemiya C, Jansen G, Neville C, Narang M, Barcelo J, O'Hoy K, Leblond S, Earle-Macdonald J, de Jong PJ, Wieringa B, and Korneluk RG. Myotonic dystrophy mutation: an unstable CTG repeat in the 3′ untranslated region of the gene. Science 255: 1253-1255, 1992.
20. Mistry DJ, Moorman JR, Reddy S, and Mounsey JP. Skeletal muscle Na currents in mice heterozygous for Six5 deficiency. Physiol Genomics 6: 153-158, 2001.
21. Moorman JR, Ackerman SJ, Kowdley GC, Griffin MP, Mounsey JP, Chen Z, Cala SE, O'Brian JJ, Szabo G, and Jones LR. Unitary anion currents through phospholemman channel molecules. Nature 377: 737-740, 1995.
22. Moorman JR, Kirsch GE, Lacerda AE, and Brown AM. Angiotensin II modulates cardiac sodium channels in neonatal rat. Circ Res 65: 1804-1804, 1989.
23. Moorman JR, Kirsch GE, VanDongen AMJ, Joho RH, and Brown AM. Fast and slow gating of sodium channels encoded by a single mRNA. Neuron 4: 243-252, 1990.
24. Mounsey JP, Helmke SM, Horne LT, John JE, Xu P, Gilbert J, Roses AD, Jones LR, Perryman MB, and Moorman JR. Phospholemman is a substrate for the human myotonic dystrophy kinase. J Biol Chem 275: 23362-23367, 2000.
25. Mounsey JP, Mistry DJ, Ai CW, Reddy S, and Moorman JR. Skeletal muscle sodium channel gating in mice deficient in myotonic dystrophy protein kinase. Hum Mol Genet 9: 2313-2320, 2000.
26. Mounsey JP, Xu P, John JE, Horne LT, Gilbert J, Roses AD, and Moorman, JR. Modulation of skeletal muscle sodium channels by human myotonin protein kinase. J Clin Invest 95: 2379-2384, 1995.
27. + channel. Circ Res 80: 370-376, 1997.
28. Nuyens D, Stengl M, Dugarmaa S, Rossenbacker T, Compernolle V, Rudy Y, Smits JF, Flameng W, Clancy CE, Moons L, Vos MA, Dewerchin M, Benndorf K, Collen D, Carmeliet E, and Carmeliet P. Abrupt rate accelerations or premature beats cause life-threatening arrhythmias in mice with long-QT3 syndrome. Nat Med 7: 1021-1027, 2001.
29. Palmer CJ, Scott BT, and Jones LR. Purification and complete sequence determination of the major plasma membrane substrate for cAMP-dependent protein kinase and protein kinase C in myocardium. J Biol Chem 266: 11126-11130, 1991.
30. Patlak JB and Ortiz M. Slow currents through single sodium channels of the adult rat heart. J Gen Physiol 86: 89-104, 1985.
31. + channels expressed in a mammalian cell line and in ventricular myocytes by protein kinase C. Proc Natl Acad Sci USA 91: 3289-3293, 1994.
32. Reddy S, Mistry D, Geddis LM, Kutchai HC, Mounsey JP, and Moorman JR. Effects of age and gene dose on skeletal muscle sodium channel gating in mice deficient in myotonic dystrophy protein kinase. Muscle Nerve 25: 850-857, 2002.
33. Reddy S, Smith DB, Rich MM, Leferovich JM, Reilly P, Davis BM, Tran K, Rayburn H, Bronson R, Cros D, Balice-Gordon RJ, and Housman D. Mice lacking the myotonic dystrophy protein kinase develop a late onset progressive myopathy. Nat Genet 13: 325-335, 1996.
34. Saba S, VanderBrink BA, Luciano B, Aronovitz MJ, Berul CI, Reddy S, Housman D, Mendelsohn ME, Estes NAM, and Wang PJ. Localization of the site of conduction abnormality in a mouse model of myotonic dystrophy. J Cardiovasc Electrophysiol 10: 1214-1220, 1999.
35. Sarkar PS, Appukuttan B, Han J, Ito Y, Ai C, Tsai W, Chai Y, Stout JT, and Reddy S. Heterozygous loss of Six5 in mice is sufficient to cause ocular cataracts. Nat Genet 25: 110-114, 2000.
36. Timchenko L, Nastainczyk W, Schneider T, Patel B, Hofmann F, and Caskey CT. Full-length myotonin protein kinase (72 kDa) displays serine kinase activity. Proc Natl Acad Sci USA 92: 5366-5370, 1995.
37. Ueda H, Shimokawa M, Yamamoto M, Kameda N, Mizusawa H, Baba T, Terada N, Fujii Y, Ohno S, Ishiura S, and Kobayashi T. Decreased expression of myotonic dystrophy protein kinase and disorganization of sarcoplasmic reticulum in skeletal muscle of myotonic dystrophy. J Neurol Sci 162: 38-50, 1999.
38. VanDongen AMJ. A new algorithm for idealizing single ion channel data containing multiple unknown conductance levels. Biophys J 70: 1303-1315, 1996.
39. + channel required for modulation by protein kinase C. Science 254: 866-868, 1991.