Myotonic dystrophy protein kinase is involved in the modulation of the Ca2+ homeostasis in skeletal muscle cells
| العنوان: | Myotonic dystrophy protein kinase is involved in the modulation of the Ca2+ homeostasis in skeletal muscle cells |
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| المؤلفون: | Jacques H. Veerkamp, A.A.G.M. Benders, Patricia J. T. A. Groenen, Frank Oerlemans, B. Wieringa |
| المصدر: | Journal of Clinical Investigation, 100, pp. 1440-1447 Journal of Clinical Investigation, 100, 1440-1447 Journal of Clinical Investigation, 100, 6, pp. 1440-1447 Journal of Clinical Investigation, 100, 1440-1447. Rockefeller univ press |
| سنة النشر: | 1997 |
| مصطلحات موضوعية: | medicine.medical_specialty, Nifedipine, Pathobiologische rol van myotone dystrofie protein kinase isovormen, Calcium-Transporting ATPases, Tetrodotoxin, Biology, Protein Serine-Threonine Kinases, Myotonic dystrophy, Myotonin-Protein Kinase, Potassium Chloride, Myotonia, Mice, Cellular ion homeostasis, Internal medicine, Het ionentransport in spiercellen van myotone dystrofie patiënten, medicine, Animals, Homeostasis, Myotonic Dystrophy, Protein kinase A, Muscle, Skeletal, GeneralLiterature_REFERENCE(e.g.,dictionaries,encyclopedias,glossaries), Cells, Cultured, Mice, Knockout, Voltage-dependent calcium channel, Myogenesis, Ryanodine, Myotonin-protein kinase, Skeletal muscle, General Medicine, medicine.disease, Acetylcholine, Mice, Inbred C57BL, Sarcoplasmic Reticulum, Muscular Atrophy, Ion transport in muscle cells of myotonic dystrophy patients, Pathobiological role of myotonic dystrophy protein kinase isoforms, Ion homeostasis, medicine.anatomical_structure, Endocrinology, Calcium, Calcium Channels, Sodium-Potassium-Exchanging ATPase, Research Article |
| الوصف: | Myotonic dystrophy (DM), the most prevalent muscular disorder in adults, is caused by (CTG)n-repeat expansion in a gene encoding a protein kinase (DM protein kinase; DMPK) and involves changes in cytoarchitecture and ion homeostasis. To obtain clues to the normal biological role of DMPK in cellular ion homeostasis, we have compared the resting [Ca2+]i, the amplitude and shape of depolarization-induced Ca2+ transients, and the content of ATP-driven ion pumps in cultured skeletal muscle cells of wild-type and DMPK[-/-] knockout mice. In vitro-differentiated DMPK[-/-] myotubes exhibit a higher resting [Ca2+]i than do wild-type myotubes because of an altered open probability of voltage-dependent l-type Ca2+ and Na+ channels. The mutant myotubes exhibit smaller and slower Ca2+ responses upon triggering by acetylcholine or high external K+. In addition, we observed that these Ca2+ transients partially result from an influx of extracellular Ca2+ through the l-type Ca2+ channel. Neither the content nor the activity of Na+/K+ ATPase and sarcoplasmic reticulum Ca2+-ATPase are affected by DMPK absence. In conclusion, our data suggest that DMPK is involved in modulating the initial events of excitation-contraction coupling in skeletal muscle. |
| وصف الملف: | application/pdf |
| اللغة: | English |
| تدمد: | 0021-9738 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ce28996b670940c7fea91ba5402bc7b5 https://hdl.handle.net/2066/28416 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....ce28996b670940c7fea91ba5402bc7b5 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 00219738 |
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