University of Wisconsin
School of Medicine and Public Health

Jonathan Makielski, MD



MADISON, WI 53705-2275

(608) 263-1172

Jonathan Makielski attended medical school at Pritzker School of Medicine, Chicago, completed his internship and residency at the University of North Carolina, Chapel Hill and pursued research and clinical fellowships at the University of Chicago. Makielski arrived at the University of Wisconsin in 1995, where he is Professor of Medicine and Physiology in the Division of Cardiovascular Medicine A Fellow of the American College of Cardiology, Makielski is board certified in Internal Medicine and in Cardiovascular Diseases.

As a clinician-investigator, Makielski has developed a distinguished research career in molecular electrophysiology. While a medical student and resident at the Universities of Chicago and North Carolina, he helped develop a computer model of the cardiac action potential. As a cardiology fellow at the University of Chicago, Makielski became interested in the then newly emerging techniques of single cell isolation and patch-clamp methods to study ion channels in single cells.

Makielski has served on multiple peer-review panels, including a the National Institutes of Health CVA, CVB, MMI, ESTA, and Surgery and Trauma Study Sections, as well as nd NIH special review (SCOR and PPG) reviews. He is presently a regularand charter member of the NHLBI training mechanisms study section.

Recognized as an excellent teacher, both in clinical and scientific settings, Makielski formally lectures to graduate students in physiology/pharmacology and toxicology. Nationally, he has participated as a lecturer in numerous courses and tutorials. He is the PI on an NIH T32 institutional training grant for Translational cardiovascular Science.

Presently he uses recombinant DNA technology and other biomolecular techniques to study cardiac ion channels. He presently has an NIH funded program to study the cardiac sodium channel, including mutations in the channel that cause arrhythmia syndromes responsible for the congenital long QT syndrome (LQT3), Brugada Syndrome, and Sudden Infant Death Syndrome (SIDS). An additional major interest funded by the NIH is the study of the ATP sensitive potassium channel (KATP) in heart and other tissues, including mitochondrial KATP using transgenic mouse models to delineate the role of KATP in ischemia and ischemic preconditioning. He has a third NIH funded project to study the role of inward rectifier potassium channels in catecholaminergic ventricular tachycardia. These basic studies have clinical implications for patients with rhythm problems.

Search Jonathan Makielski's literature abstracts on PubMed



Ye B, Kroboth SL, Pu J-L, Sims JJ, Aggarwal N, McNally EM, Makielski JC, Shi N-Q. Molecular Identification and Functional Characterization of a Mitochondrial Sulfonylurea Receptor 2 Splice Variant Generated by Intraexonic Splicing 2009 Circ Res.105:

Vega, AL, Tester, DJ, Ackerman MJ, Makielski, JC Protein Kinase A–Dependent Biophysical Phenotype for V227F-KCNJ2 Mutation in Catecholaminergic Polymorphic Ventricular Tachycardia C Circ Arrhythm Electrophysiol. 2009 Oct;2(5):540-7

Valdivia CR, Ueda K, Ackerman MJ, Makielski JC. Gpd1l Links Redox State To Cardiac Excitability By PKC-Dependent Phosphorylation Of The Na Channel Scn5a. Am J Physiol Heart Circ Physiol. 2009. Oct;297(4):H1446-52.

Ueda K, Valdivia CR, Medeiros-Domingo A, Tester DJ, Vatta M, Farrugia G, Ackerman MJ, Makielski JC. Syntrophin mutation associated with long QT syndrome through activation of the nNOS-SCN5A macromolecular complex PNAS 105:9355-9360 2008.

Pu JL, Ye B, Kroboth SL, McNally EM, Makielski JC, Shi NQ. Cardiac sulfonylurea receptor short form-based channels confer a glibenclamide-insensitive K(ATP) activity. J Mol Cell Cardiol. 44 188–200 2008

Van Norstrand DW, Valdivia CR, Tester DJ, Ueda K, London B, Makielski JC, Ackerman MJ. Molecular and functional characterization of novel glycerol-3-phosphate dehydrogenase 1 like gene (GPD1-L) mutations in sudden infant death syndrome. Circulation 2007;116:2253-2259

Tan B-Ha, Iturralde-Torres P, Medeiros-Domingo A, Nava S, Tester DJ, Valdivia CR, Tusié-Luna T, Ackerman MJ, Makielski JC A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder and ventricular tachycardia Cardiovasc Res 76:409-417 2007

Eckhardt LL, Farley AL, Rodriguez E, Ruwaldt K, Hammill D., Tester DJ, Ackerman MJ, Makielski JC KCNJ2 mutations in arrhythmia patients referred for LQT testing: a mutation T305A with novel effect on rectification properties. Heart Rhythm 4:323-329 2007.