Recent studies have shed more light onto the genetical basis of familial hemiplegic migraine (FHM) as a channelopathy due to 3 abnormal loci of the brain specific P/Q type calcium channel alpha 1A subunit gene (CACNA1A) on chromosome 1 and 19p13 [1]. CACNA1A regulates the release of various neurotransmitters, probably including serotonin. Circulatory changes detected by different imaging techni...

Mutations in the skeletal muscle voltage-gated calcium channel (CaV1.1) have been associated with hypokalemic periodic paralysis, but how the pathogenesis of this disorder relates to the functional consequences of mutations was unclear. In this issue of the JCI, Wu and colleagues recapitulate the disease by generating a novel knock-in CaV1.1 mutant mouse and use this model to investigate the ce...

Inherited channelopathies are at the origin of many neurological disorders. Here we report a form of channelopathy that is acquired in experimental temporal lobe epilepsy (TLE), the most common form of epilepsy in adults. The excitability of CA1 pyramidal neuron dendrites was increased in TLE because of decreased availability of A-type potassium ion channels due to transcriptional (loss of chan...

Hemiplegic migraine (HM) is an unusual subset of migraine with aura, in which headache is associated with unilateral motor deficits, thought to be attributable to an underlying calcium channelopathy. In some cases the neurological dysfunction may outlast the headache and persist for many days. In the initial stages, hemiplegic migraine may mimic cerebral infarction. Within the first few hours a...

PURPOSE Familial hypokalemic periodic paralysis (HOKPP) is an autosomal dominant channelopathy characterized by episodic attacks of muscle weakness and hypokalemia. Mutations in the calcium channel gene, CACNA1S, or the sodium channel gene, SCN4A, have been found to be responsible for HOKPP; however, the mechanism that causes hypokalemia remains to be determined. The aim of this study was to im...