[Omega pore, an alternative ion channel permeation pathway involved in the development of several channelopathies].
Voltage gated ion channels (VGIC) constitute a large family of ion channels. VGIC are responsible for ions to cross the membrane. They are composed of a pore domain associated to voltage sensor domains (VSD), which regulate the function of the pore. The VSD has been recognized as the unit responsible for sensing electrical signals of all VGIC. Recently, mutations within the VSD have been studied and revealed the creation of a new permeation pathway directly through the usually non-conductive VSD. This new permeation pathway has been called omega pore or gating pore. Given the number, the diversity and the large roles of VSD, gating pores might become an important pathological defect. Indeed, several mutations have been associated to the development of several pathologies such as periodic paralysis, arrhythmias and cardiac dilatation or also the peripheral nerve hyperexcitability.
Centre de recherche de l'Institut universitaire en santé mentale de Québec, 2601 chemin de la Canardière, Québec, QC, G1J 2G3 Canada.
Adrien
A
Moreau
Centre de recherche de l'Institut universitaire en santé mentale de Québec, 2601 chemin de la Canardière, Québec, QC, G1J 2G3 Canada - Département de médecine, université Laval, Québec, QC, G1K 7P4 Canada.
Mohamed
M
Chahine
D000818:Animals; D053447:Channelopathies / Q000209:etiology*; D006801:Humans; D007473:Ion Channels / Q000502:physiology*
D007473:Ion Channels