• Mutations in the genes encoding a calcium channel (Ca V 1.1) and a sodium channel (Na V 1.4) have been identified in HypoPP families. (jci.org)
  • Genes encoding voltage-gated calcium, sodium and potassium channels that likely contribute to E/I balance have been implicated in autism. (sfari.org)
  • There are a myriad of mutations identified in genes encoding cardiac transcription factors, ion channels, gap junctions, energy metabolism regulators, lamins and other structural proteins. (medscape.com)
  • These rare diseases are often the underlying cause of sudden cardiac death in young individuals and result from mutations in several genes encoding ion channels or proteins involved in their regulation. (cdc.gov)
  • Ion channels can be classified by different properties, including by the gating mechanism or the ion selectivity of the pore. (tocris.com)
  • Ryanodex (dantrolene sodium) is a skeletal muscle relaxant used to treat malignant hyperthermia in conjunction with appropriate supportive measures, and to prevent malignant hyperthermia in patients at high risk. (rxlist.com)
  • Our Ryanodex (dantrolene sodium) Side Effects Drug Center provides a comprehensive view of available drug information on the potential side effects when taking this medication. (rxlist.com)
  • RYANODEX® (dantrolene sodium) for injectable suspension is a sterile lyophilized powder. (rxlist.com)
  • RYANODEX is supplied in 20 mL vials containing 250 mg dantrolene sodium and the following inactive ingredients: 125 mg mannitol, 25 mg polysorbate 80, 4 mg povidone K12 and sufficient sodium hydroxide or hydrochloric acid for pH adjustment. (rxlist.com)
  • Divalproex sodium has been approved as adjunctive therapy for patients with multiple seizure types that include absence seizures. (medscape.com)
  • Divalproex sodium is indicated for monotherapy or adjunctive therapy in both focal-onset and generalized-onset seizures and adjunctively in many seizure types, including absence seizures. (medscape.com)
  • Depakote (divalproex sodium) dissociates to the valproate ion in the gastrointestinal tract. (centerwatch.com)
  • For example RyR1 is activated following membrane depolarization of skeletal muscle, whereas depolarization of cardiac muscle results in Ca 2+ influx through L-type Ca 2+ channels, which activates RyR2 by CICR. (tocris.com)
  • Incidentally the depolarization-induced activation of RyR1 channels in skeletal muscle is dependent on a physical interaction between RyR1 and L-type Ca 2+ channels. (tocris.com)
  • Temperature-sensitive mutations in the III-IV cytoplasmic loop region of the skeletal muscle sodium channel gene in paramyotonia congenita. (xenbase.org)
  • The rate of intravenous fosphenytoin sodium injection administration should not exceed 150 mg phenytoin sodium equivalents (PE) per minute in adults and 2 mg PE/kg/min (or 150 mg PE/min, whichever is slower) in pediatric patients because of the risk of severe hypotension and cardiac arrhythmias. (nih.gov)
  • Fosphenytoin sodium injection, USP can also be substituted, as short-term use, for oral phenytoin. (nih.gov)
  • Fosphenytoin sodium injection, USP should be used only when oral phenytoin administration is not possible. (nih.gov)
  • DILANTIN is available as 30 mg and 100 mg extended phenytoin sodium capsules. (nih.gov)
  • The alpha subunit is a tetramer which forms the pore, the voltage sensor, and the calcium sensing region. (wikipedia.org)
  • Medical records of an 8-year-old boy with PEM were analyzed retrospectively, which included clinical characteristics, follow-up information, and SCN9A (Sodium Voltage-Gated Channel Alpha Subunit 9) gene analysis. (frontiersin.org)
  • A rat brain Na+ channel alpha subunit with novel gating properties. (xenbase.org)
  • Familial pain syndromes from mutations of the NaV1.7 sodium channel. (nih.gov)
  • The voltage gated Na channels Nav1.7, Nav1.8 and Nav1.9 were found to be selectively up-regulated in rat DRG neurons treated with GM-CSF, which resulted in enhanced excitability. (iasp-pain.org)
  • To address the question of specificity for the allele encoding the Na V 1.4-R669H variant as a cause of HypoPP and to produce a model system in which to characterize functional defects of the mutant channel and susceptibility to paralysis, we generated knockin mice carrying the ortholog of the gene encoding the Na V 1.4-R669H variant (referred to herein as R669H mice). (jci.org)
  • Disorders resulting from a mutation in the gene encoding an ion channel or its regulatory proteins are called channelopathies. (tocris.com)
  • Human genetic studies have identified mutations in the sodium channel SCN5A gene causing tachyarrhythmia disorders, as well as progressive cardiac conduction system diseases, or overlapping syndromes. (medscape.com)
  • Changes in sodium channel gating produced by point mutations in a cytoplasmic linker. (xenbase.org)
  • voltage-gated ion channel Any ion channel that opens and closes in response to changes in electrical potential across the cell membrane in which the channel is situated. (encyclopedia.com)
  • In humans they are divided into three subtypes: large conductance or BK channels, which have very high conductance which range from 100 to 300 pS, intermediate conductance or IK channels, with intermediate conductance ranging from 25 to 100 pS, and small conductance or SK channels with small conductances from 2-25 pS. (wikipedia.org)
  • Subtypes of BK Channels KCa1.1 (BK, Slo1, Maxi-K, KCNMA1) Beta subunits: β1, β2, β3, β4 Intermediate conductance channels seem to be the least studied of all of the channels. (wikipedia.org)
  • Structurally they are thought to be very similar to BK channels with the main differences being conductance, and the methods of modulation. (wikipedia.org)
  • Destruction of sodium conductance inactivation in squid axons perfused with pronase. (xenbase.org)
  • Destruction of the sodium conductance inactivation by a specific protease in perfused nerve fibres from Loligo. (xenbase.org)
  • IK channels have shown a strong connection to calcification in vasculature, as inhibition of the channel causes a decrease in vascular calcification. (wikipedia.org)
  • This imbalance in calcium metabolism occurs because calcium mobilization from bone into the serum pool is insufficient to compensate for the efflux of calcium via the mammary glands during lactation. (merckvetmanual.com)
  • After Luo-Rudy passive model was published in 1991 by updating INa (sodium current) and IK, IK1, IKp but keeping the calcium current ICa same as Beeler and Reuter's, Dr. Rudy heard in the annual heart conferences that almost all researchers were very happy about the robustness of Luo-Rudy passive model as used to investigate the clinic phenomena. (scholarpedia.org)
  • In 1991, the most arguing issue to formulate a live cardiac cell model was the ambiguity of the calcium ion channels. (scholarpedia.org)
  • Covers selective permeability of membranes, diffusion, and facilitated diffusion (including channels and carrier proteins). (khanacademy.org)
  • This domain is found in sodium, potassium, and calcium ion channels proteins. (embl.de)
  • In some Na channel proteins the domain is repeated four times, whereas in others (e.g. (embl.de)
  • Surprisingly, however, whereas metazoan bestrophin channels are anion-selective and activated by Ca 2+ , KpBest is cation-selective and does not contain the Ca 2+ sensor region (the 'Ca 2+ clasp') that is conserved amongst metazoan organisms. (elifesciences.org)
  • There are several types of voltage-gated channel, each allowing the selective passage of a particular ion. (encyclopedia.com)
  • ZLCS ) , a biopharmaceutical company that discovers and develops novel treatments for patients suffering from pain, today announced that it has initiated the second of two Phase 2a clinical studies with Z160, its first-in-class, oral, state-dependent, selective N-type (Cav2.2) calcium channel blocker for the potential treatment of chronic neuropathic pain. (aol.com)
  • Advanced Z160, a first-in-class, oral, state dependent, selective N-type calcium channel (Cav 2.2) blocker into two Phase 2a clinical trials for neuropathic pain including lumbosacral radiculopathy (LSR) which began in the third quarter of 2012 and postherpetic neuralgia which began in the fourth quarter of 2012. (aol.com)
  • Internal perfusion with a fast sodium channel blocker eliminated spontaneous bursting but did not block the LTS. (nih.gov)
  • Z944 is a novel, oral, T-type calcium channel blocker which has demonstrated efficacy in a number of preclinical inflammatory pain models and other disease models. (aol.com)
  • Below is a list of the 8 known human calcium-activated potassium channel grouped according to sequence homology of transmembrane hydrophobic cores: Though not implied in the name, but implied by the structure these channels can also be activated by voltage. (wikipedia.org)
  • Biophysical and molecular mechanisms of Shaker potassium channel inactivation. (xenbase.org)
  • One of the first studies to elucidate how the pulsed electromagnetic frequencies could affect human health showed that low-intensity pulsed electromagnetic frequencies could be blocked using drugs that block voltage-gated calcium channels. (news-medical.net)
  • A new, important, medical problem (that seemed temporally related to cessation of fluoride exposure and subsequent negative calcium balance) was renal calculus formation, with stones composed of calcium oxalate. (fluoridealert.org)
  • Subsequent studies showed activation of calcium channels in response to pulsed electromagnetic frequencies in plants, animals, and human cells. (news-medical.net)
  • Recent evidence suggests that the early calcium wave elicited by an axonal injury induces epigenetic changes in the nucleus, thereby priming the system for subsequent transcriptional events. (nature.com)
  • In dogs, supplementation with oral calcium during pregnancy may predispose the animal to eclampsia during peak lactation, because excessive calcium intake during pregnancy causes downregulation of the calcium regulatory system and subsequent clinical hypocalcemia when calcium demand is high. (merckvetmanual.com)
  • Careful cardiac monitoring is needed during and after administering intravenous fosphenytoin sodium injection. (nih.gov)
  • The syndrome is caused by changes in the structure and function of certain cardiac ion channels and reduced expression of Connexin 43 (Cx43) in the Right Ventricle (RV), predominantly in the Right Ventricular Outflow Tract (VSVD), causing electromechanical abnormalities. (bvsalud.org)
  • The identified CHRNA2 mutation changes a single protein building block (amino acid) in the α2 subunit of nAChR channels. (medlineplus.gov)
  • This mutation makes the channels more sensitive to the neurotransmitter acetylcholine, allowing them to open more easily than usual. (medlineplus.gov)
  • These highlights do not include all the information needed to use FOSPHENYTOIN SODIUM INJECTION safely and effectively. (nih.gov)
  • See full prescribing information for FOSPHENYTOIN SODIUM INJECTION. (nih.gov)
  • Fosphenytoin sodium injection, USP is indicated for the treatment of generalized tonic-clonic status epilepticus and prevention and treatment of seizures occurring during neurosurgery. (nih.gov)
  • Unlike other channels, opening of the pore is due to the repositioning of tethered pore-lining helices within a surrounding protein shell that dramatically widens a neck of the pore through a concertina of amino acid rearrangements. (elifesciences.org)
  • A cluster of hydrophobic amino acid residues required for fast Na(+)-channel inactivation. (xenbase.org)
  • A neutral amino acid change in segment IIS4 dramatically alters the gating properties of the voltage-dependent sodium channel. (xenbase.org)
  • Amino acid residues required for fast Na(+)-channel inactivation: charge neutralizations and deletions in the III-IV linker. (xenbase.org)
  • During 2012, we made a number of advances with our novel ion channel programs, including advancing our novel formulation of Z160 into Phase 2 clinical development and advancing Z944 into the clinic,' commented Mark H.N. Corrigan, MD, President and CEO of Zalicus. (aol.com)
  • The ryanodine receptors (RyRs) are a family of Ca 2+ channels that mediate the release of Ca 2+ from intracellular Ca 2+ storage organelles. (tocris.com)
  • A molecular view of neurotransmitter receptors and ionic channels. (xenbase.org)
  • Furthermore RyR phosphorylation is thought to induce channel opening, a mechanism that may cause SR calcium leak in the heart. (tocris.com)
  • Downregulation in cellular ion homeostasis, including ion channel function and neurotransmitter release, were observed after knocking out AEG‑1 expression. (spandidos-publications.com)
  • Hundreds of different types of ion channel have been identified in the membranes of all biological cells. (tocris.com)
  • Here, we showed that GM-CSF promotes bone cancer-associated pain by enhancing excitability of DRG neurons via the Jak2-Stat3-mediated upregulation of expression of nociceptor-specific voltage-gated sodium channels. (iasp-pain.org)
  • The linker between the S5 and S6 region serves to form the pore of the channel. (wikipedia.org)
  • The channel comprises five BEST1 subunits arranged symmetrically around a central ion pore that is ~95 Å long. (elifesciences.org)
  • Knowing the structure of these channels can provide insight into their function and mechanism of gating. (wikipedia.org)
  • It is proposed that the hydrophobic cluster of Ile-1488, Phe-1489, and Met-1490 serves as a hydrophobic latch that stabilizes the inactivated state in a hinged-lid mechanism of Na(+)-channel inactivation. (xenbase.org)
  • Ion channels are essential for life and play a fundamental role in physiological processes such as muscle contraction and nutrient transport. (tocris.com)
  • other medications targeting these channels are under study for the treatment of schizophrenia , Alzheimer's disease , and pain. (medlineplus.gov)
  • The beta subunit of the channel is thought to be a regulatory subunit of the channel. (wikipedia.org)
  • Partly because bestrophin channels have no sequence or structural similarity to other ion channels, the molecular mechanisms underlying gating are unknown. (elifesciences.org)
  • Structural parts involved in activation and inactivation of the sodium channel. (xenbase.org)
  • These findings indicate that BK channels are involved in the relaxation of smooth muscle cells. (wikipedia.org)
  • This finding has sparked further exploration surrounding these channels and researchers have found that IK channels regulate the cell cycle in cancer cells, B and T lymphocytes, and stem cells. (wikipedia.org)
  • These results suggest that persistent sodium channels are essential for spontaneous burst generation in ET cells. (nih.gov)
  • Cromolyn Sodium Inhalation Solution acts by inhibiting the release of chemicals from your mast cells by indirectly blocking calcium channels from entering the mast cells. (northwestpharmacy.com)
  • The physiological effects of BK channels have been studied extensively using knockout mice. (wikipedia.org)
  • Primary structure and functional expression of the beta 1 subunit of the rat brain sodium channel. (xenbase.org)
  • H Heyne, D Baez-Nieto et al, Predicting functional effects of missense variants in voltage-gated sodium and calcium channels, Sci Transl Med;12(556):eaay6848. (lu.se)