Paramyotonia congenita, the major characteristics of which are cold-induced and exercise-induced myotonia, is an autosomal-dominant muscle disease which is classified into one of a group of muscle diseases, so-called muscle "sodium channelopathies" caused by missense mutations in the gene coding for the skeletal muscle sodium channel a-subunit (SCN4A) (1-4). Such muscle sodium channelopathies s...

The functional properties of most sodium channels are too similar to permit identification of specific sodium channel types underlying macroscopic current. Such discrimination would be particularly advantageous in the nervous system in which different sodium channel family isoforms are coexpressed in the same cell. To test whether members of the mu-conotoxin family can discriminate among known ...

Mexiletine as the first choice drug in myotonia treatment is a chiral sodium channel blocker clinically used in its racemic form. The phenolic structure of this drug, prompted us to design its novel calix[4]arene-based cluster in a chalice-shaped structure. Therefore, the present study reports the synthesis and in-vitro anti-myotonic activity of the chalice-shaped cluster of mexiletine...

Cardiac sodium channels are integral membrane proteins whose structure is not known at atomic level yet and their molecular kinetics is still being studied through mathematical modeling. This study has focused on adapting an existing model of cardiac Na channel to analyze molecular kinetics of channels at 9-37°C. Irvine et al developed a Markov model for Na channel using Neuronal Network Model ...

  Objective(s): Recently, phthalimide derivatives were designed based on ameltolide and thalidomide as they possess a similar degree of anticonvulsant potency due to their phenytoin-like profile. The ability of phthalimide pharmacophore to interact with neuronal voltage-dependent sodium channels was studied in the batrachotoxin affinity assay. Therefore, in the present study, a series of 19 com...

Cardiac sodium channels are integral membrane proteins whose structure is not known at atomic level yet and their molecular kinetics is still being studied through mathematical modeling. This study has focused on adapting an existing model of cardiac Na channel to analyze molecular kinetics of channels at 9-37°C. Irvine et al developed a Markov model for Na channel using Neuronal Network Model ...

Action potentials (APs) are initiated in the proximal axon of most neurons. In myelinated axons, a 50-times higher sodium channel density in the initial segment compared to the soma may account for this phenomenon. However, little is known about sodium channel density and gating in proximal unmyelinated axons. To study the mechanisms underlying AP initiation in unmyelinated hippocampal mossy fi...

Hypertensives of African origin have low-renin, sodium-sensitive blood pressure and respond poorly to treatment with angiotensin converting enzyme inhibitors. The epithelial sodium channel may be important in the pathogenesis of essential hypertension in this population. This is supported by the identification of mutations within this channel, which lead to excess sodium reabsorption and hypert...

AIM Dysnatremic cases are frequently faced in clinical practice and the macroscopic effects of it and its consequences are well known, but microscopic effects of it are not well defined, this research tries to reveal the effects of dysnatremia at the cellular level. MATERIAL AND METHODS By using an action potential simulation, the effects of the extracellular sodium concentration on the sodiu...