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Effects of age and gene dose on skeletal muscle sodium channel gating in mice deficient in myotonic dystrophy protein kinase
Author(s) -
Reddy Sita,
Mistry Dilaawar J.,
Wang Qing Cai,
Geddis Lisa M.,
Kutchai Howard C.,
Moorman J. Randall,
Mounsey J. Paul
Publication year - 2002
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.10127
Subject(s) - myotonic dystrophy , myotonia , gating , skeletal muscle , muscular dystrophy , endocrinology , medicine , sodium channel , protein kinase a , myopathy , electrophysiology , kinase , biology , chemistry , microbiology and biotechnology , sodium , neuroscience , organic chemistry
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