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Na v 1.4 slow‐inactivation: Is it a player in the warm‐up phenomenon of myotonic disorders?
Author(s) -
Lossin Christoph
Publication year - 2013
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.23713
Subject(s) - phenomenon , medicine , psychology , neuroscience , physics , quantum mechanics
Myotonia is a heritable disorder in which patients are unable to willfully relax their muscles. The physiological basis for myotonia lies in well‐established deficiencies of skeletal muscle chloride and sodium conductances. What is unclear is how normal muscle function can temporarily return with repeated movement, the so‐called “warm‐up” phenomenon. Electrophysiological analyses of the skeletal muscle voltage‐gated sodium channel Na v 1.4 (gene name SCN4A ), a key player in myotonia, have revealed several parallels between the Na v 1.4 biophysical signature, specifically slow‐inactivation, and myotonic warm‐up, which suggest that Na v 1.4 is critical not only in producing the myotonic reaction, but also in mediating the warm‐up. Muscle Nerve, 2013