Bjorn C Knollmann
Affiliation: Vanderbilt University
Gómez Hurtado N, Boczek N, Kryshtal D, Johnson C, Sun J, Nitu F, et al
. Novel CPVT-Associated Calmodulin Mutation in CALM3 (CALM3-A103V) Activates Arrhythmogenic Ca Waves and Sparks. Circ Arrhythm Electrophysiol. 2016;9: pubmed publisher
..A small proportion of A103V-CaM is sufficient to evoke arrhythmogenic Ca disturbances via ryanodine receptor 2 dysregulation, which explains the autosomal dominant inheritance. ..
Chavali N, Kryshtal D, Parikh S, Wang L, Glazer A, Blackwell D, et al
. The patient-independent human iPSC model - a new tool for rapid determination of genetic variant pathogenicity in long QT syndrome. Heart Rhythm. 2019;: pubmed publisher
..Furthermore, our results indicate the importance of controlling beating rates to evaluate functional significance of LQTS VUS in high-throughput hiPSC-CM assays. ..
Walweel K, Gómez Hurtado N, Rebbeck R, Oo Y, Beard N, Molenaar P, et al
. Calmodulin inhibition of human RyR2 channels requires phosphorylation of RyR2-S2808 or RyR2-S2814. J Mol Cell Cardiol. 2019;130:96-106 pubmed publisher
..Rather, CaM's biological action on human RyR2 appears to be more nuanced, with inhibitory activity only on phosphorylated RyR2 channels, which occurs during exercise or in patients with heart failure. ..
Faggioni M, van der Werf C, Knollmann B. Sinus node dysfunction in catecholaminergic polymorphic ventricular tachycardia: risk factor and potential therapeutic target?. Trends Cardiovasc Med. 2014;24:273-8 pubmed publisher
..Herein, we review the pathophysiology of CPVT and discuss the role of sinus node dysfunction as a modulator of arrhythmia risk and potential therapeutic target. ..
Hwang H, Kryshtal D, Feaster T, SÃ¡nchez Freire V, Zhang J, Kamp T, et al
. Comparable calcium handling of human iPSC-derived cardiomyocytes generated by multiple laboratories. J Mol Cell Cardiol. 2015;85:79-88 pubmed publisher
..We conclude that hiPSC-CMs generated independently from multiple iPSC lines using monolayer-based methods can be reproducibly recovered from cryopreservation and exhibit comparable and functional SR Ca handling. ..
Knollmann B, Chopra N, Hlaing T, Akin B, Yang T, Ettensohn K, et al
. Casq2 deletion causes sarcoplasmic reticulum volume increase, premature Ca2+ release, and catecholaminergic polymorphic ventricular tachycardia. J Clin Invest. 2006;116:2510-20 pubmed
Kryshtal D, Gryshchenko O, Gómez Hurtado N, Knollmann B. Impaired calcium-calmodulin-dependent inactivation of Cav1.2 contributes to loss of sarcoplasmic reticulum calcium release refractoriness in mice lacking calsequestrin 2. J Mol Cell Cardiol. 2015;82:75-83 pubmed publisher
..2, which contributes to the loss of SR Ca(2+) release refractoriness in the Casq2 KO mouse model and, therefore, may further increase risk for ventricular arrhythmia in vivo. ..
Kryshtal D, Dawling S, Seger D, Knollmann B. In Vitro Studies Indicate Intravenous Lipid Emulsion Acts as Lipid Sink in Verapamil Poisoning. J Med Toxicol. 2016;12:165-71 pubmed publisher
..Our in vitro studies indicate that ILE acts as a lipid sink that rapidly reverses impaired cardiomyocyte contractility in the continued presence of verapamil. ..
Parikh S, Blackwell D, Gómez Hurtado N, Frisk M, Wang L, Kim K, et al
. Thyroid and Glucocorticoid Hormones Promote Functional T-Tubule Development in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes. Circ Res. 2017;121:1323-1330 pubmed publisher
..This new hormone maturation method could advance the use of hiPSC-CM for disease modeling and cell-based therapy. ..