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Publication : Effects of age and gene dose on skeletal muscle sodium channel gating in mice deficient in myotonic dystrophy protein kinase.

First Author  Reddy S Year  2002
Journal  Muscle Nerve Volume  25
Issue  6 Pages  850-7
PubMed ID  12115974 Mgi Jnum  J:116209
Mgi Id  MGI:3693171 Doi  10.1002/mus.10127
Citation  Reddy S, et al. (2002) Effects of age and gene dose on skeletal muscle sodium channel gating in mice deficient in myotonic dystrophy protein kinase. Muscle Nerve 25(6):850-7
abstractText  Myotonic muscular dystrophy (DM) is characterized by abnormal skeletal muscle Na channel gating and reduced levels of myotonic dystrophy protein kinase (DMPK). Electrophysiological measurements show that mice deficient in Dmpk have reduced Na currents in muscle. We now find that the Na channel expression level is normal in mouse muscle partially or completely deficient in Dmpk. Reduced current amplitudes are not changed by age or gene dose, and the reduction is not due to changes in macroscopic or microscopic gating kinetics. The mechanism of abnormal membrane excitability in DM may in part be silencing of muscle Na channels due to Dmpk deficiency.
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