Effects of age and gene dose on skeletal muscle sodium channel gating in mice deficient in myotonic dystrophy protein kinase
✍ Scribed by Sita Reddy; Dilaawar J. Mistry; Qing Cai Wang; Lisa M. Geddis; Howard C. Kutchai; J. Randall Moorman; J. Paul Mounsey
- Publisher
- John Wiley and Sons
- Year
- 2002
- Tongue
- English
- Weight
- 264 KB
- Volume
- 25
- Category
- Article
- ISSN
- 0148-639X
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✦ Synopsis
Abstract
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. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25: 000–000, 2002