ATAXIA, ARRHYTHMIA AND ION-CHANNEL GENE DEFECTS

Ion channels are essential to a wide range of physiological functions including neuronal signaling, muscle contraction cardiac pacemaking, h ormone secretion and cell proliferation The important role that highly regulated ion influx plays In these processes has been underscored by a recent flurry of discovered linking ion-channel gene mutations to i nherited disorders. Ion channels of many, different types hare been de monstrated as being causative factors. In genetic disease This review discusses the growing number of disorders associated with genes of the voltage-gated ion channel superfamily with special focus on those cha racterized by neurological, neuromuscular, or cardiac dysfunction in h umans and mice.