Human RYR2 (Ryanodine receptor 2) ELISA Kit

We report a 17-year-old boy with a large RYR2 exon 3 deletion who has a severe catecholaminergic polymorphic ventricular tachycardia (CPVT) phenotype characterized by refractoriness to both nadolol and flecainide which has previously not been reported in this subgroup of CPVT patients. Treatment options in a patient like ours are therefore limited and sympathectomy and implantable cardioverter-defibrillator implantation should be considered early in the treatment course as was done in this patient. In contrast to other CPVT patients who do not usually have structural cardiac abnormalities, these patients are at a high risk of developing left ventricular noncompaction or dilated cardiomyopathy and therefore might benefit from cardiac imaging at regular intervals.

Fuzi, the processed lateral roots of Aconitum carmichaelii Debx., is a traditional herbal medicine that is well known for its excellent pharmacological effects and acute toxicity. Aconitine is one of the diester-diterpene alkaloids and well-known for its arrhythmogenic effects. However, the effects of aconitine in zebrafish have rarely been studied. Therefore, we investigated the effects of aconitine on zebrafish embryos and H9c2 cells. Zebrafish embryos at 48 hours postfertilization were exposed to aconitine, and then, cardiac function and apoptosis were measured. Through transcriptomic analysis, the cardiotoxicity of aconitine in zebrafish embryos was involved in regulating Ca2+ signal pathways. A reverse transcription-polymerase chain reaction was performed to verify the expression of Ca2+ pathway-related genes after 12, 24, 36 and 48 hours of treatment. Meanwhile, intracellular Ca2+ concentrations and cell apoptosis were observed in H9c2 cells treated with half-maximal inhibitory concentration values of aconitine for 30 minutes. The protein levels of troponin T (TnT), caspase 3, Bcl-2 and Bax were detected by western blot analysis. In vivo, 2.0 and 8.0 mu m aconitine decreased the heart rate and inhibited the contraction of ventricles and atria in a dose- and time-dependent manner. Furthermore, aconitine increased expression of cacna1c, RYR2, atp2a2b, Myh6, troponin C, p38, caspase 3, Bcl-2 and Bax for 12 hours. In vitro, 1.5 and 4.5 mm aconitine caused intracellular Ca2+ ion oscillation, increased rates of apoptosis, inhibited TnT and Bcl-2 protein expression, and promoted caspase 3 and Bax protein expression. These data confirmed that aconitine at various concentrations induced cardiac dysfunction and apoptosis were related to the Ca2+ signaling pathway.