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Calcium entry activated by ablation of both JP45 (zeige JSRP1 ELISA Kits)-CASQ1 (zeige CASQ1 ELISA Kits) and JP45 (zeige JSRP1 ELISA Kits)-CASQ2 complexes supports tetanic force development in slow twitch soleus muscles.
The minimal KEKE motif of TRN (zeige TNPO1 ELISA Kits) involved in the interactions with CSQ2, HRC (zeige HRC ELISA Kits) and RyR2 (zeige RYR2 ELISA Kits) within the Ca2 (zeige CA2 ELISA Kits)+ release units of cardiac sarcoplasmic reticulum has been defined.
While CASQ2 stabilizes RyR2 (zeige RYR2 ELISA Kits) rendering it refractory in the diastolic phase, HRC (zeige HRC ELISA Kits) enhances RyR2 (zeige RYR2 ELISA Kits) activity facilitating RyR2 (zeige RYR2 ELISA Kits) recovery from refractoriness.
Results show that CASQ2 deletion causes abnormal sarcoplasmic reticulum Ca(2 (zeige CA2 ELISA Kits)+) release and selective interstitial fibrosis in the atrial pacemaker complex, which disrupt SAN pacemaking and increase susceptibility to atrial fibrillation
Viral gene transfer of wild-type CASQ2 into the heart of R33Q mice prevents and reverts severe manifestations of catecholaminergic polymorphic ventricular tachycardia.
Cardiac hypertrophy and calcium waves in CASQ2-null cardiac muscle are governed by the ryanodine receptor (Ryr)2 Ca2+ sensor.
Expression of CASQ2-R33Q influences molecular and ultra-structural heart development; post-natal, adaptive changes appear capable of ensuring until adulthood a new pathophysiological equilibrium.
Mutant CASQ2(D307H) protein retains some of its physiological function. Its expression decreases with age and is inversely related to arrhythmia severity.
Qualitatively similar results were obtained in a hybrid strain created by crossing CASQ2 knockout mice with mice deficient in phospholamban (zeige PLN ELISA Kits).
The triadin (zeige TRDN ELISA Kits)-to-calsequestrin ratio is a critical modulator of the sarcoplasmic reticulum Ca(2 (zeige CA2 ELISA Kits)+) signaling in ventricular myocytes.
a direct interaction exists between RyR2 (zeige RYR2 ELISA Kits) and CSQ2, is reported.
induced Pluripotent Stem Cell-derived cardiomyocytes are useful for investigating the similarities/differences in the pathophysiological consequences of RyR2 (zeige RYR2 ELISA Kits) versus CASQ2 mutations underlying Catecholaminergic polymorphic ventricular tachycardia.
Mutations in the MYBPC3 (zeige MYBPC3 ELISA Kits) and CASQ2 genes and six combinations between loci in the MYBPC3 (zeige MYBPC3 ELISA Kits), MYH7 (zeige MYH7 ELISA Kits) and CASQ2 genes were responsible for cardiomyopathy risk in a studied cohort.
We observed association between a CASQ2 polymorphism and SCA due to VA in patients with CAD (zeige CAD ELISA Kits) adjusting for CHF and independent associations between CASQ2 SNPs and CHF adjusting for SCA.
The sarcoplasmic reticulum calcium content in human type II fibres is primarily determined by the CSQ1 (zeige CASQ1 ELISA Kits) abundance, and in type I fibres, by the combined amounts of both CSQ1 (zeige CASQ1 ELISA Kits) and CSQ2.
Molecular analysis of the CASQ2 gene in 43 probands with Catecholaminergic polymorphic ventricular tachycardia were performed and eight mutations in five patients, were identified.
In a consanguineous family, a novel homozygous CASQ2 mutation (p.L77P) was identified in a child with CPVT who required implantation of a cardioverter defibrillator due to episodes of syncope while on medical therapy
A review of the physiology of Casq2 in cardiac Ca2 (zeige CA2 ELISA Kits)+ handling and discuss pathophysiological mechanisms that lead to catecholaminergic polymorphic ventricular tachycardia caused by CASQ2 mutations.
patients with CASQ2-associated CPVT should be recommended to receive ICDs to prevent sudden death when medical therapy is not effective.
Aspartate to histidine casq2 mutation causes arrhythmia in cardiomyocytes generated from catecholaminergic polymorphic ventricular tachycardia patients.
endogenous ankyrin repeat domain 1 protein and CASQ2 are co-enriched in piglet cardiac Purkinje cells
data shows that phosphorylated calsequestrin is required for high capacity calcium buffering and suggest that ryanodine receptor (zeige RYR3 ELISA Kits) inhibition by calsequestrin is mediated by junctin (zeige ASPH ELISA Kits)
The protein encoded by this gene specifies the cardiac muscle family member of the calsequestrin family. Calsequestrin is localized to the sarcoplasmic reticulum in cardiac and slow skeletal muscle cells. The protein is a calcium binding protein that stores calcium for muscle function. Mutations in this gene cause stress-induced polymorphic ventricular tachycardia, also referred to as catecholaminergic polymorphic ventricular tachycardia 2 (CPVT2), a disease characterized by bidirectional ventricular tachycardia that may lead to cardiac arrest.
calsequestrin 2 (cardiac muscle)
, calsequestrin, cardiac muscle isoform
, cardiac calsequestrin
, calsequestrin 2, fast-twitch, cardiac muscle
, SR calcium binding protein
, calsequestrin homologue
, calsequestrin, skeletal muscle isoform
, laminin-binding protein