Referenced in: none

Automatically associated channels: Nav1.4 , Slo1

Title: Nav 1.4 slow-inactivation: is it a player in the warm-up phenomenon of myotonic disorders?

Authors: Christoph Lossin

Journal, date & volume: Muscle Nerve, 2013 Apr , 47, 483-7

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/23381896

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.