Potassium voltage gated channel subfamily. Medical search. Frequent questions

This gene encodes a member of the potassium channel, voltage-gated, subfamily H. This member is a pore-forming (alpha) subunit of a voltage-gated non-inactivating delayed rectifier potassium channel. (wikipedia.org)
One beta subunit, made from a KCNE protein, attaches (binds) to the channel and regulates its activity. (medlineplus.gov)
L-type voltage-dependent calcium channel alpha subunit 1C is a novel candidate gene associated with secondary hyperparathyroidism: an application of haplotype-based analysis for multiple linked single nucleotide polymorphisms. (genscript.com)
Homo sapiens potassium voltage-gated channel subfamily A regulatory beta subunit 2 (KCNAB2), transcript variant 2, mRNA. (ucsc.edu)
Accessory potassium channel protein which modulates the activity of the pore-forming alpha subunit. (ucsc.edu)
Belongs to the shaker potassium channel beta subunit family. (ucsc.edu)
Here I investigate the effects of subunit composition on toxin blocking affinity, using three members of the Shaker subfamily of potassium channel α-subunits (Kv1.1, Kv1.2 and Kv1.4), which are known to form heteromultimers in vivo, in the Xenopus oocyte expression system. (aspetjournals.org)
The single subunit model, in which a single toxin-sensitive subunit renders the channel toxin sensitive, best described all of the data for the two dendrotoxins and the block of tityustoxin-Kα for a mixed population of channels composed of Kv1.1 and Kv1.2 subunits. (aspetjournals.org)
The data suggest that subunit composition of potassium channels can have a large effect on toxin block and that different toxins yield strikingly diverse results with the same pair of subunits, even when they are nearly identical in blocking affinity for the toxin-sensitive subunit. (aspetjournals.org)
Voltage-gated potassium channel subunit beta-1 or Kvβ1.1 is encoded by the gene KCNAB1. (antibodiesinc.com)
Kvβ1.1 is a cytoplasmic subunit that modulates the characteristics of the membrane spanning, channel-forming alpha-subunits. (antibodiesinc.com)
Voltage-gated potassium channel subunit beta-1 (EC 1.1.1. (antibodiesinc.com)
The targets of the epilepsy pipeline drugs market are Gamma-Aminobutyric Acid Type A Receptor Subunit (GABA, Voltage Gated Sodium Channel, Cannabinoid Receptor 2, Cannabinoid Receptor 1, Potassium Voltage Gated Channel Subfamily KQT Member 2, Potassium Voltage Gated Channel Subfamily KQT Member 3, Sodium Channel Protein Type 8 Subunit Alpha, Sodium Channel Protein Type 1 Subunit Alpha, 5-Hydroxytryptamine Receptor, and Serine/Threonine Protein Kinase mTOR. (globaldata.com)
Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. (scbdd.com)
Channel properties are modulated by cAMP and subunit assembly. (scbdd.com)

Four sequence-related potassium channel genes - shaker, shaw, shab, and shal - have been identified in Drosophila, and each has been shown to have human homolog(s). (nih.gov)
This gene encodes a member of the potassium channel, voltage-gated, shaker-related subfamily. (ucsc.edu)
Using the crystal structure of the Aplysia Shaker channel tetramerization domain as a template, the common structure of the POZ domain class was predicted. (embl.de)
Crystal structure of the tetramerization domain of the Shaker potassium channel. (embl.de)
We have determined the crystal structure of the T1 domain of a Shaker potassium channel at 1.55 A resolution. (embl.de)
Kvß1.1 is a member of the potassium channel, voltage-gated, shaker-related subfamily. (antibodiesinc.com)
This gene encodes a voltage-gated delayed potassium channel that is phylogenetically related to the Drosophila Shaker channel. (thermofisher.com)

This member is a delayed rectifier potassium channel and its activity is modulated by some other family members. (nih.gov)
Predicted to enable delayed rectifier potassium channel activity. (mcw.edu)

Homo sapiens potassium inwardly rectifying channel subfamily J member 14 (KCNJ14), mRNA. (genscript.com)
Inwardly rectifying potassium (Kir) channels open at the 'helix bundle crossing' (HBC), formed by the M2 helices at the cytoplasmic end of the transmembrane pore. (nature.com)
Note that whilst the 'Potassium channels' family is listed under 'Voltage-gated ion channels' two of the subfamilies, the Inwardly rectifying potassium channels and Two P domain potassium channels, are NOT voltage-gated. (guidetopharmacology.org)

This gene encodes a member of a family of single pass transmembrane domain proteins that function as ancillary subunits to voltage-gated potassium channels. (nih.gov)
Four alpha subunits made from KCNQ1 proteins form the structure of each channel. (medlineplus.gov)
Functional expression of inward rectifier potassium channels in cultured human pulmonary smooth muscle cells: evidence for a major role of Kir2.4 subunits. (genscript.com)
Voltage-dependent, ion-selective channels such as Na+, Ca2+ and K+ channel proteins function as tetrameric assemblies of identical or similar subunits. (embl.de)
It is known to confer specificity for tetramer formation, so the heteromeric assembly of K+-channel subunits is an important mechanism for the observed channel diversity. (embl.de)
channel properties depend on the type of alpha subunits that are part of the channel (By similarity). (nih.gov)
Channel properties are modulated by cytoplasmic beta subunits that regulate the subcellular location of the alpha subunits and promote rapid inactivation (By similarity). (nih.gov)
Kv3 channel subunits encoded by Kcnc genes are distinguished by rapid activation and deactivation kinetics that support high-frequency, narrow action potential firing. (jneurosci.org)
Large, non-GABAergic DCN projection neurons express Kv3.1 and Kv3.3 channel subunits. (jneurosci.org)
The ability of voltage-gated potassium channel α-subunits to form heteromultimers has complicated efforts to use toxins to characterize potassium channels in native cells. (aspetjournals.org)
Beta subunits can promote the closure of channels or enhance channel activity depending on the channel members. (antibodiesinc.com)
The N-terminus of the channel is associated with beta subunits that can modify the inactivation properties of the channel as well as affect expression levels. (thermofisher.com)

Potassium voltage-gated channel subfamily H member 1 is a protein that in humans is encoded by the KCNH1 gene. (wikipedia.org)
The specific function of a potassium channel depends on its protein components and its location in the body. (medlineplus.gov)
The KCNQ1 protein interacts with proteins in the KCNE family (such as the KCNE1 protein) to form functional potassium channels. (medlineplus.gov)
In cardiac muscle cells, the mutations appear to increase the flow of potassium ions through the channel formed with the KCNQ1 protein. (medlineplus.gov)
Most of these changes lead to the production of an abnormally short, nonfunctional version of the KCNQ1 protein that cannot be used to build potassium channels. (medlineplus.gov)
Other mutations change a small number of amino acids in this protein, which alters the normal structure and function of the channels. (medlineplus.gov)
These changes allow the protein to form channels but reduce the channels' ability to transport potassium ions out of cardiac muscle cells. (medlineplus.gov)
The mutations alter the function of ion channels made with the KCNQ1 protein, increasing the channels' activity. (medlineplus.gov)
The protein encoded by this gene is an integral membrane protein and inward-rectifier type potassium channel, and probably has a role in controlling the excitability of motor neurons. (genscript.com)
KCNQ1OT1 , or potassium voltage-gated channel subfamily Q member 1 opposite transcript 1 is the antisense transcript of the protein-coding gene KCNQ1 . (medscape.com)
Known to be a protein-protein interaction motif found at the N-termini of several C2H2-type transcription factors as well as Shaw-type potassium channels. (embl.de)
The C-terminus of the channel is complexed to a PDZ domain protein that is responsible for channel targeting. (thermofisher.com)
Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a potassium-selective channel through which potassium ions may pass in accordance with their electrochemical gradient. (icr.ac.uk)

Predicted to be involved in potassium ion transmembrane transport. (mcw.edu)
Voltage-gated potassium channel that mediates transmembrane potassium transport in excitable membranes. (nih.gov)
Kir channels consist of only two transmembrane helices plus an extensive C-terminal cytoplasmic tail of the channel pore. (nature.com)

These channels, which transport positively charged atoms (ions) of potassium out of cells, play key roles in a cell's ability to generate and transmit electrical signals. (medlineplus.gov)
An inability of these channels to properly transport potassium ions in the inner ear and cardiac muscle leads to the hearing loss and arrhythmia characteristic of Jervell and Lange-Nielsen syndrome. (medlineplus.gov)
As a result, more potassium ions flow out of cardiac muscle cells at a critical time during the heartbeat, which can lead to an irregular heart rhythm. (medlineplus.gov)
Forms tetrameric potassium-selective channels through which potassium ions pass in accordance with their electrochemical gradient (PubMed:2347305, PubMed:14575698). (nih.gov)
Introduced negative charges at the HBC (G178D) in Kir2.2 channels forces opening, allowing pore wetting and free movement of permeant ions between the cytoplasm and the inner cavity. (nature.com)

This gene encodes a member of the potassium channel, voltage-gated, shab-related subfamily. (nih.gov)
We report a case of congenital LQT2 with potassium voltage-gated channel subfamily H member 2 gene ( KCNH2 ) mutation misdiagnosed as refractory epilepsy and treated with various AEDs for 22 years. (biomedcentral.com)
Diagnosis of LQTS type 2 (LQT2) was confirmed by ECG and the detection of potassium voltage-gated channel subfamily H member 2 gene ( KCNH2 ) mutation and epilepsy was ruled out and all AEDs were withdrawn based on her normal 24-h video electroencephalograph (VEEG) monitoring. (biomedcentral.com)

Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr). (scbdd.com)
Mediates voltage-dependent potassium ion permeability of excitable membranes. (icr.ac.uk)

The KCNH1 gene encodes a highly conserved voltage-gated potassium channel with predominant expression in the adult central nervous system. (wikipedia.org)
They then went on to demonstrate that de novo missense mutations in the KCNH1 gene cause deleterious gain of function in the voltage-gated potassium channel, resulting in the multisystem developmental disorder. (wikipedia.org)
The KCNQ1 gene belongs to a large family of genes that provide instructions for making potassium channels. (medlineplus.gov)
BACKGROUND: Mutations in the cardiac sodium channel gene SCN5A cause Brugada syndrome (BrS), an arrhythmic disorder that is a leading cause of sudden death and lacks effective treatment. (bvsalud.org)

We reported the crystal structure of a chicken Kir2.2[G178D] mutant channel 12 , in which the introduced G178D mutations at the HBC functionally stabilize the open conformation, a strategy used previously to obtain an open crystal structure of a bacterial homolog KirBac3.1 13 . (nature.com)

Associates with KCNQ2 or KCNQ5 to form a potassium channel with essentially identical properties to the channel underlying the native M-current, a slowly activating and deactivating potassium conductance which plays a critical role in determining the subthreshold electrical excitability of neurons as well as the responsiveness to synaptic inputs. (calixar.com)
The delayed rectifier potassium conductance in the sarcolemma and the transverse tubular system membranes of mammalian skeletal muscle fibers. (uni-bielefeld.de)
Ion channel gating and conductance are classically understood as separate processes. (nature.com)
The present data reveal how individual protonation events change the electrostatic microenvironment of the pore, resulting in step-wise alterations of ion pooling, and hence conductance, that appear as 'gated' substates. (nature.com)
In the G178D structure, the HBC gate is slightly wider than in previous structures, and molecular dynamics (MD) simulations demonstrate rapid wetting of the G178D pore at the HBC region, followed by further expansion and K + conductance through the channel. (nature.com)

Channels made with KCNQ1 proteins are primarily found in the inner ear and in heart (cardiac) muscle. (medlineplus.gov)
Starting from native material or recombinant systems, we succeed with all types of membrane proteins: GPCRs, Ion Channels, Transporters, Receptors and Viral Proteins. (calixar.com)
CALIXAR's approach allows to preserve the original structure and function of membrane proteins (GPCRs, Ion Channels, Transporters, Receptors, Anchors and Viral Proteins) providing solutions for pharmaceutical industries, biotechnology companies and academic teams to develop conformational antibodies, formulate new vaccines, carry out Structure Based Drug Discovery and/or HTS assays. (calixar.com)
Using iterative database searches, a statistically significant sequence similarity was detected between the POZ (poxvirus and zinc finger) domains found in a variety of proteins involved in animal transcription regulation, cytoskeleton organization, and development, and the tetramerization domain of animal potassium channels. (embl.de)

Previous work has shown that the Shal potassium channel subfamily underlies the predominant fast transient outward current in Drosophila neurons (Tsunoda, S., and Salkoff, L. (1995) J. Neurosci. (wikigenes.org)

Moreover, BrS iPSC-CMs showed cardiac sodium channel (Nav1.5) loss-of-function as compared to control iPSC-CMs. (bvsalud.org)

Voltage-gated potassium (Kv) channels represent the most complex class of voltage-gated ion channels from both functional and structural standpoints. (nih.gov)
Potassium channels represent the most complex class of voltage-gated ion channels from both functional and structural standpoints. (prosci-inc.com)
The functional channel is a homotetramer. (thermofisher.com)

Vullhorst D, Klocke R, Bartsch JW, Jockusch H. Expression of the potassium channel KV3.4 in mouse skeletal muscle parallels fiber type maturation and depends on excitation pattern. (uni-bielefeld.de)

A synthetic peptide corresponding to a sequence at the C-terminus of human Kv1.1 potassium channel (465-481aa IAHYRQVNIRTANCTTA). (thermofisher.com)

Isoforms USO have no channel activity by themself, but modulates channel characteristics by forming heterotetramers with other isoforms which are retained intracellularly and undergo ubiquitin-dependent degradation. (scbdd.com)

Members of this family affect diverse processes in potassium channel regulation, including ion selectivity, voltage dependence, and anterograde recycling from the plasma membrane. (nih.gov)

Homotetrameric channels display rapid activation and slow inactivation (PubMed:2347305). (nih.gov)

KCNE4 and KCNE5: K(+) channel regulation and cardiac arrhythmogenesis. (nih.gov)
The patient has never been suspected LQTS because her normal routine 12 channel electrocardiograph (ECG) at early disease stage, until we caught clinical attacks during her initial visit to our emergency room (ER) and documented simultaneous cardiac arrhythmia. (biomedcentral.com)
In the heart, the channels are involved in recharging the cardiac muscle after each heartbeat to maintain a regular rhythm. (medlineplus.gov)
FINDINGS: D356Y iPSC-CMs exhibited increased beat interval variability, slower depolarization, cardiac arrhythmias, defects of Na+ channel function and irregular Ca2+ signaling, when compared to controls. (bvsalud.org)

Bulk Order Inquiry for Anti-Kvbeta1.1 K+ Channel Antibody FL490 Conjugate ------- (please add any order requirements, including desired quantity, timing, etc. (antibodiesinc.com)

Potassium channels are present in all types of cells and play critical roles in control of multiple physiological processes. (nature.com)

Single-channel recordings reveal striking, pH-dependent, subconductance behaviors in G178D (or G178E and equivalent Kir2.1[G177E]) mutant channels, with well-resolved non-cooperative subconductance levels. (nature.com)

SHL-1 currents strongly resembled Shal currents in other species except that they were active in a more depolarized voltage range. (wikigenes.org)

Downregulation in cellular ion homeostasis, including ion channel function and neurotransmitter release, were observed after knocking out AEG‑1 expression. (spandidos-publications.com)

The channel alternates between opened and closed conformations in response to the voltage difference across the membrane (PubMed:2347305, PubMed:14575698). (nih.gov)

The channel activity is up-regulated by cAMP. (icr.ac.uk)
Interaction with KCN4B/POMP is necessary for the modulation of channel activity by cAMP. (icr.ac.uk)

These 4 subfamilies are placed in a single category based on overall function and evolutionary relationships. (guidetopharmacology.org)

Previously we reported that increased lateral deviation while ambulating and slips while traversing a narrow beam of ataxic Kcnc3 -null mice were corrected by restoration of Kv3.3 channels specifically to Purkinje cells, whereas Kcnc3 -mutant mice additionally lacking one Kcnc1 allele were partially rescued. (jneurosci.org)

Fold prediction and evolutionary analysis of the POZ domain: structural and evolutionary relationship with the potassium channel tetramerization domain. (embl.de)

She had head computed tomography (CT), 24-h VEEG, 12-channel dynamic ECG and blood analyses. (biomedcentral.com)
In the present study, we carry out detailed single-channel analyses of cKir2.2[G178D] and hKir2.1[G177E] channels that reveal striking sub-state gating behavior. (nature.com)

Predicted to act upstream of or within potassium ion transport. (mcw.edu)

Predicted to be part of voltage-gated potassium channel complex. (mcw.edu)
SHL-1 channels are apparently important in fine-tuning complex behaviors, such as mating, that play a crucial role in the survival and propagation of the species. (wikigenes.org)

The role of the voltage-gated potassium channel, Kv2.1 in prostate cancer cell migration. (nih.gov)
The role of cerebellar Kv3.1 and Kv3.3 channels in motor coordination was examined with an emphasis on the deep cerebellar nuclei (DCN). (jneurosci.org)

KCNE Regulation of K(+) Channel Trafficking - a Sisyphean Task? (uni-bielefeld.de)

Potassium channels are present in most mammalian cells, where they participate in a wide range of physiologic responses. (genscript.com)

Kv3 channels are distinguished by rapid activation and deactivation, which confer upon them the ability to rapidly repolarize action potentials. (jneurosci.org)

Anatomy26

Organisms17

Diseases4

Chemicals and Drugs148

Analytical, Diagnostic and Therapeutic Techniques and Equipment12

Phenomena and Processes42

Disciplines and Occupations4

Technology, Industry, Agriculture1

Information Science4

Subunit15

Shaker7

Rectifier potassium channel2

Inwardly3

Subunits12

Protein13

Transmembrane3

Ions5

Member3

Mediates2

Gene4

Mutations1

Conductance5

Proteins4

Transient outward1

Sodium Channel1

Functional3

Kv3.41

Kv1.11

Modulates1

Selectivity1

Inactivation1

Cardiac4

Conjugate1

Processes1

Kir2.11

Shal1

Expression1

Membrane1

Activity2

Function1

Previously1

Domain1

Analyses2

Transport1

Complex2

Role2

Regulation1

Present1

Ability1