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, 47 (4), 483-7

Nav 1.4 Slow-Inactivation: Is It a Player in the Warm-Up Phenomenon of Myotonic Disorders?

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Nav 1.4 Slow-Inactivation: Is It a Player in the Warm-Up Phenomenon of Myotonic Disorders?

Christoph Lossin. Muscle Nerve.

Abstract

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 Nav 1.4 (gene name SCN4A), a key player in myotonia, have revealed several parallels between the Nav 1.4 biophysical signature, specifically slow-inactivation, and myotonic warm-up, which suggest that Nav 1.4 is critical not only in producing the myotonic reaction, but also in mediating the warm-up.

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