Arrhythmias

Arrhythmias are a consistent and potentially life-threatening manifestation in TANGO2-related metabolic encephalopathy and arrhythmias, an autosomal recessive disorder caused by mutations in the TANGO2 gene. This condition is characterized by recurrent metabolic crises featuring rhabdomyolysis, cardiac arrhythmias, and neurodegeneration, with onset typically in childhood presenting as acute encephalomyopathic symptoms including hypotonia, neurologic regression, and delayed psychomotor development. Progressive neurodegeneration leads to epilepsy, cognitive impairment, pyramidal and cerebellar signs, and loss of expressive language, while cardiac involvement manifests as severe arrhythmias, contributing significantly to morbidity and mortality.

References:

[1]. Metabolic Crises, Recurrent, with Rhabdomyolysis, Cardiac Arrhythmias, and Neurodegeneration (MECRCN). malacards.

Arrhythmias (36):

Targets:	Potassium Channel (13) Sodium Channel (9) Calcium Channel (6) Adrenergic Receptor (2) Drug Derivative (2) ATP Synthase (1) Adenosine Receptor (1) Autophagy (1) Bradykinin Receptor (1) CaMK (1) Drug Metabolite (1) HIV (1) Myosin (1) Na+/K+ ATPase (1) Opioid Receptor (1) Platelet-activating Factor Receptor (PAFR) (1) Prostaglandin Receptor (1)

Cat. No.	Product Name	CAS No.	Purity	Chemical Structure

HY-14187

Amiodarone	1951-25-3	98.32%
Amiodarone, a benzofuran-based Class III antiarrhythmic agent, inhibits WT outward ionic current (IhERG) tails with an IC₅₀ of ∼45 nM. Amiodarone induces cell proliferation and myofibroblast differentiation via ERK1/2 and p38 MAPK signaling in fibroblasts. Amiodarone can be used in the research of both supraventricular and ventricular arrhythmias.

HY-P1604

ATX-II	60748-45-0
ATX-II is a selective sodium channel modulator toxin. ATX-II enhances late sodium current, prevents full sodium channel inactivation, and generates persistent current fractions. ATX-II has pro-arrhythmic effect. ATX-II slows intrinsic heart rate, prolongs QT interval and sinus node recovery time, and causes sinus pauses and arrests. ATX-II can be used for the research of atrial fibrillation, long QT syndrome, and long QT3 syndrome.

HY-P1604A

ATX-II TFA		99.65%
ATX-II TFA is a selective sodium channel modulator toxin. ATX-II TFA enhances late sodium current, prevents full sodium channel inactivation, and generates persistent current fractions. ATX-II TFA has pro-arrhythmic effect. ATX-II TFA slows intrinsic heart rate, prolongs QT interval and sinus node recovery time, and causes sinus pauses and arrests. ATX-II TFA can be used for the research of atrial fibrillation, long QT syndrome, and long QT3 syndrome.

HY-149662

TMDJ-035	2681302-83-8	99.92%
TMDJ-035 is a high-affinity, selective RyR2 inhibitor with an EC₅₀ of 0.0130 μM. TMDJ-035 reduces RyR2 protein expression without affecting action potential-induced Ca²⁺ transients. TMDJ-035 decreases ATP content and intracellular Ca²⁺ levels. TMDJ-035 inhibits arrhythmias in a CPVT mouse model carrying mutant RyR2s. TMDJ-035 has no effect on electrocardiogram parameters or cardiac systolic function. TMDJ-035 exacerbates heart failure in mouse myocardial infarction models and hypoxic cardiomyocytes by altering cardiac function, causing tissue damage, promoting inflammatory infiltration, collagen deposition, and changes in Myosin heavy chain/actin expression. TMDJ-035 can be used in studies related to heart failure, catecholaminergic polymorphic ventricular tachycardia, and arrhythmias.

HY-136661

(-)-N6-Phenylisopropyl adenosine	38594-96-6	99.45%
(-)-N6-phenylisopropyl adenosine (L-Phenylisopropyladenosine) is a selective A1 adenosine receptor agonist. (-)-N6-phenylisopropyl adenosine inhibits K⁺-induced Ca²⁺ uptake with an IC₅₀ value of 0.5 µM. (-)-N6-phenylisopropyl adenosine protects against ischemia-induced ventricular arrhythmias and atrial fibrillation, and exacerbates ethanol withdrawal symptoms. (-)-N6-phenylisopropyl adenosine also has analgesic effects.

HY-165443

Cassaine	468-76-8
Cassaine (Nervocidine) is a Na⁺/K⁺-ATPase inhibitor. Cassaine stabilizes phosphorylated intermediates, slows spontaneous dephosphorylation and blocks potassium-stimulated dephosphorylation. Cassaine exhibits non-competitive inhibition with respect to K⁺, shows inhibition enhanced by Pi and antagonized by Na⁺. Cassaine induces positive inotropic effects in perfused hearts with faster inotropy offset. Cassaine can be used for research on cardiac arrhythmias.

HY-182646

NE-10133	149889-02-1
NE-10133 is a I_SK and I_KS potassium channel (Potassium Channel) inhibitor. NE-10133 inhibits voltage-dependent and slowly activated delayed rectifier potassium currents. NE-10133 exhibits class III antiarrhythmic activity. NE-10133 is applicable for research related to arrhythmias.

HY-147256A

Cavutilide hydrochloride	1276021-10-3
Cavutilide hydrochloride (Niferidil) is a class III antiarrhythmic agent that inhibits hERG K⁺ channel. Cavutilide hydrochloride has the potential for the study of persistent atrial fibrillation.

HY-108592

UCL 2077	918311-87-2	98.91%
UCL 2077 is a potassium channel and slow afterhyperpolarization (sAHP) inhibitor. UCL 2077 selectively blocks sAHP channels without affecting L-type Ca²⁺ currents. UCL 2077 blocks KCNQ1- and KCNQ2-containing K⁺ channels, decreases erg current amplitude, increases erg deactivation rate. UCL 2077 can be used for the research of hippocampus-dependent memory retrieval deficit and cardiac arrhythmias.

HY-110140

(E)-KN-93 phosphate	1188890-41-6	99.85%
(E)-KN-93 phosphate is a Ca²⁺/calmodulin-dependent protein kinase II (CaMKII) inhibitor with IC₅₀ values of 9 μM and 3 μM against CaMKIIδ_Monomer and CaMKIIδ_Dodecamer, respectively. (E)-KN-93 phosphate can be used in research on epilepsy, depression, and arrhythmias..

HY-N3198A

Sophoramine	6882-66-2	99%
Sophoramine ((-)-Sophoramine) is a prejunctional α₂-adrenoceptor inhibitor that can be found in the seeds of Sophora alopecuroides. Sophoramine inhibits prejunctional α2-adrenoceptors to facilitate norepinephrine release from adrenergic nerves. Sophoramine shows cytotoxic activity against human cancer cells, positive inotropic activity, and activity in rodent arrhythmia models. Sophoramine can be used for the research of arrhythmias.

HY-113040A

(17R,18S)-Epoxyeicosatetraenoic acid	725246-18-4
(17R,18S)-Epoxyeicosatetraenoic acid (17 (R),18 (S)-EETeTr) is a physiologically active fatty acid metabolite and also a vasodilator targeting BKα. (17R,18S)-Epoxyeicosatetraenoic acid activates the outward potassium current mediated by BK channels, and this effect is independent of the BKβ1 subunit, intracellular/extracellular calcium levels, and sarcoplasmic reticulum calcium release regulated by RyR3. (17R,18S)-Epoxyeicosatetraenoic acid is produced by the epoxidation of eicosapentaenoic acid mediated by CYP1A1 variants. (17R,18S)-Epoxyeicosatetraenoic acid is applicable to research related to arrhythmia.

HY-118387

AVE-0118	498577-53-0	98.93%
AVE-0118 is a Kv1.5 potassium channel blocker and antiarrhythmic agent. AVE-0118 blocks neuronal Kv1.5 potassium channels, thereby enhancing the release of norepinephrine. AVE-0118 enhances field stimulation-induced neurogenic contraction, an effect sensitive to α1-adrenergic receptor blockade. AVE-0118 terminates persistent atrial fibrillation in some dogs. AVE-0118 is applicable to research related to atrial fibrillation and persistent atrial fibrillation.

HY-184190

ERG-IN-7
ERG-IN-7 is a Nav1.5, Cav1.2 and Kv11.1 inhibitor with IC₅₀ values of 10.34 μM, 21.01 μM, and 16.385 μM for human Nav1.5, Cav1.2, and Kv11.1, respectively. ERG-IN-7 prolongs action potentials, reduces conduction velocity, and restores cardiac function. ERG-IN-7 can be used for the research of ventricular arrhythmia.

HY-184207

Zinc13732787	119486-83-8
Zinc13732787 is an inhibitor of the KCNQ1/KCNE1 potassium channel complex, with no activity against the KCNQ2/KCNQ3 channel. Zinc13732787 reduces axonal growth in human neural stem cells, while exerting no effect on KCNQ1-knockout neural stem cells. Zinc13732787 can be used for research on arrhythmia, epilepsy, and neuropsychiatric disorders.

HY-182334

azo-Q2a
azo-Q2a is a photoswitchable Na_V1.5 inhibitor. azo-Q2a exists as the trans isomer with low activity in the dark or under 480 nm illumination, and exists as the cis isomer with higher inhibitory potency under 365 nm illumination. azo-Q2a reduces the heart rate of living zebrafish larvae in a light-dependent manner. azo-Q2a can be used for the study of arrhythmias.

HY-106501A

Asocainol hydrochloride	91574-89-9
Asocainol hydrochloride (Goe 4704 hydrochloride) is an antiarrhythmic agent. Asocainol hydrochloride reduces the maximum rate of action potential rise and action potential amplitude. Asocainol hydrochloride is applicable for the research of arrhythmias.

HY-181063

Nav1.5-IN-1	899207-91-1
Nav1.5-IN-1 is a selective Nav1.5 inhibitor with an IC₅₀ of 1.38 μM. Nav1.5-IN-1 shows selectivity over other Nav subtypes. Nav1.5-IN-1 reduces cardiac conduction in isolated rat hearts.Nav1.5-IN-1 can be used for the research of arrhythmias.

HY-118945

FR191413	194928-55-7
FR191413 is a selective bradykinin B2 receptor agonist that stimulates prostaglandin E2 production in WI-38 cells and activates BK B2 receptor-mediated pathways such as vasodilation and organ protection. FR191413 competitively binds to [³H]-BK in guinea pig ileum (GPI) membranes and CHO cells transfected with the human BK B2 receptor, with IC₅₀ values of 20.0 nM and 2.60 nM, respectively. FR191413 can be used in research related to cardiovascular diseases and diabetes, including hypertension, myocardial hypertrophy, myocardial infarction, arrhythmias, and diabetic nephropathy.

HY-180335

L 691121 free base	136075-61-1
L 691121 (free base) is the major metabolite of L 691121 in plasma. L 691121 (free base) is a class IIIantiarrhythmic agent that blocks potassium currents.

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