Mouse Anti-SCN5A Recombinant Antibody (CBXS-5091) (CBMAB-S2310-CQ)

Mouse

Human

CBXS-5091

IgM

ICC, IF, WB

Synthetic peptide corresponding to a portion of the extracellular domain of Nav1.5

Human

IgM

Monoclonal

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Liquid

PBS, pH 7.2

0.9 mg/mL

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Sodium Voltage-Gated Channel Alpha Subunit 5

The protein encoded by this gene is an integral membrane protein and tetrodotoxin-resistant voltage-gated sodium channel subunit. This protein is found primarily in cardiac muscle and is responsible for the initial upstroke of the action potential in an electrocardiogram. Defects in this gene are a cause of long QT syndrome type 3 (LQT3), an autosomal dominant cardiac disease. Alternative splicing results in several transcript variants encoding different isoforms.

Sodium Voltage-Gated Channel Alpha Subunit 5; Sodium Channel, Voltage-Gated, Type V, Alpha Subunit; Sodium Channel Protein Cardiac Muscle Subunit Alpha; Voltage-Gated Sodium Channel Subunit Alpha Nav1.5; HH1; Cardiac Tetrodotoxin-Insensitive Voltage-Dependent Sodium Channel Alpha Subunit; Sodium Channel, Voltage-Gated, Type V, Alpha (Long QT Syndrome 3); Sodium Channel, Voltage Gated, Type V Alpha Subunit; Sodium Channel Protein Type 5 Subunit Alpha; Sodium Channel Protein Type V Subunit Alpha; Long QT Syndrome 3; Nav1.5; PFHB1;

This protein mediates the voltage-dependent sodium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a sodium-selective channel through which Na+ ions may pass in accordance with their electrochemical gradient (PubMed:1309946, PubMed:21447824, PubMed:25370050, PubMed:23420830, PubMed:23085483, PubMed:26279430, PubMed:26392562, PubMed:26776555).
It is a tetrodotoxin-resistant Na+ channel isoform (PubMed:1309946).
This channel is responsible for the initial upstroke of the action potential. Channel inactivation is regulated by intracellular calcium levels (PubMed:19074138).

Biological Process atrial cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process AV node cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process AV node cell to bundle of His cell communicationManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process brainstem developmentISS:BHF-UCL
Biological Process bundle of His cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process cardiac conduction system development1 PublicationNAS:BHF-UCL
Biological Process cardiac muscle cell action potential involved in contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process cardiac muscle contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process cardiac ventricle developmentISS:BHF-UCL
Biological Process cellular response to calcium ionManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process cerebellum developmentISS:BHF-UCL
Biological Process membrane depolarizationManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process membrane depolarization during action potentialManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process membrane depolarization during atrial cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process membrane depolarization during AV node cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process membrane depolarization during bundle of His cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process membrane depolarization during cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process membrane depolarization during Purkinje myocyte cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process membrane depolarization during SA node cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process neuronal action potentialManual Assertion Based On ExperimentIBA:GO_Central
Biological Process odontogenesis of dentin-containing toothISS:BHF-UCL
Biological Process positive regulation of action potentialISS:BHF-UCL
Biological Process positive regulation of epithelial cell proliferationISS:BHF-UCL
Biological Process positive regulation of sodium ion transportManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process regulation of atrial cardiac muscle cell membrane depolarizationManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process regulation of atrial cardiac muscle cell membrane repolarizationManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process regulation of cardiac muscle cell contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process regulation of heart rateManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process regulation of heart rate by cardiac conductionManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process regulation of sodium ion transmembrane transportManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process regulation of ventricular cardiac muscle cell membrane depolarizationManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process regulation of ventricular cardiac muscle cell membrane repolarizationManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process response to denervation involved in regulation of muscle adaptationISS:BHF-UCL
Biological Process SA node cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process sodium ion transmembrane transportManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process sodium ion transportManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process telencephalon developmentISS:BHF-UCL
Biological Process ventricular cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL

Cell membrane
Cytoplasm, perinuclear region
Cell membrane, sarcolemma, T-tubule
RANGRF promotes trafficking to the cell membrane.

Progressive familial heart block 1A (PFHB1A):
A cardiac bundle branch disorder characterized by progressive alteration of cardiac conduction through the His-Purkinje system, with a pattern of a right bundle-branch block and/or left anterior hemiblock occurring individually or together. It leads to complete atrio-ventricular block causing syncope and sudden death.
Long QT syndrome 3 (LQT3):
A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy.
Brugada syndrome 1 (BRGDA1):
A tachyarrhythmia characterized by right bundle branch block and ST segment elevation on an electrocardiogram (ECG). It can cause the ventricles to beat so fast that the blood is prevented from circulating efficiently in the body. When this situation occurs, the individual will faint and may die in a few minutes if the heart is not reset.
Sick sinus syndrome 1 (SSS1):
The term 'sick sinus syndrome' encompasses a variety of conditions caused by sinus node dysfunction. The most common clinical manifestations are syncope, presyncope, dizziness, and fatigue. Electrocardiogram typically shows sinus bradycardia, sinus arrest, and/or sinoatrial block. Episodes of atrial tachycardias coexisting with sinus bradycardia ('tachycardia-bradycardia syndrome') are also common in this disorder. SSS occurs most often in the elderly associated with underlying heart disease or previous cardiac surgery, but can also occur in the fetus, infant, or child without heart disease or other contributing factors. SSS1 onset is in utero, infancy, or early childhood.
Familial paroxysmal ventricular fibrillation 1 (VF1):
A cardiac arrhythmia marked by fibrillary contractions of the ventricular muscle due to rapid repetitive excitation of myocardial fibers without coordinated contraction of the ventricle and by absence of atrial activity.
Sudden infant death syndrome (SIDS):
SIDS is the sudden death of an infant younger than 1 year that remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene, and review of clinical history. Pathophysiologic mechanisms for SIDS may include respiratory dysfunction, cardiac dysrhythmias, cardiorespiratory instability, and inborn errors of metabolism, but definitive pathogenic mechanisms precipitating an infant sudden death remain elusive.
Atrial standstill 1 (ATRST1):
A rare arrhythmia characterized by the absence of electrical and mechanical activity in the atria. Electrocardiographically, it is characterized by bradycardia, the absence of P waves, and a junctional narrow complex escape rhythm.
Cardiomyopathy, dilated 1E (CMD1E):
A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Atrial fibrillation, familial, 10 (ATFB10):
A familial form of atrial fibrillation, a common sustained cardiac rhythm disturbance. Atrial fibrillation is characterized by disorganized atrial electrical activity and ineffective atrial contraction promoting blood stasis in the atria and reduces ventricular filling. It can result in palpitations, syncope, thromboembolic stroke, and congestive heart failure.

Ubiquitinated by NEDD4L; which promotes its endocytosis. Does not seem to be ubiquitinated by NEDD4 or WWP2.
Phosphorylation at Ser-1503 by PKC in a highly conserved cytoplasmic loop slows inactivation of the sodium channel and reduces peak sodium currents (Probable). Regulated through phosphorylation by CaMK2D (By similarity).
Lacks the cysteine which covalently binds the conotoxin GVIIJ. This cysteine (position 868) is speculated in other sodium channel subunits alpha to be implied in covalent binding with the sodium channel subunit beta-2 or beta-4.