Jonathan C. Makielski, MD, FACC Print Friendly Page
Faculty, University of Wisconsin School of Medicine and Public HealthA Fellow of the American College of Cardiology, Dr. Makielski is board certified in internal medicine and in the subspecialty of cardiovascular diseases (cardiology). Recognized as an excellent teacher both in clinical and scientific settings, Dr. Makielski formally lectures to graduate students in physiology, genetics, pharmacology and toxicology. Nationally, he has participated as a lecturer in numerous courses and tutorials. As a clinician-investigator, Dr. Makielski has developed a distinguished NIH-funded research career in molecular electrophysiology.
Specialties
UW Health Clinics
Hospital Affiliation(s)
University of Wisconsin Hospital and Clinics (primary)
Languages Spoken
| English |
| Medical interpreters are available to help patients communicate with hospital and clinic staff. For more information, please contact interpreter services at (608) 262-9000. |
UW School of Medicine and Public Health
| Department of Medicine |
Professional Certifications and Education
| Board Certification |
Internal Medicine
Cardiovascular Disease |
| Fellowship |
University of Chicago Medical Center, Chicago, IL
|
| Residency |
North Carolina Memorial Hospital
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| Internship |
North Carolina Memorial Hospital
|
| Medical School |
University of Chicago, Chicago, IL, 1979
|
Programs & Conditions
| Research |
Dr. Makielski 's research has been continuously funded by the NIH since 1985 and he is a leader in ion channels research employing molecular biological and electrophysiological methods. His interests include the genetic basis of arrhythmia, such as the Burgada syndrome and long QT syndrome, with a focus on the cardiac sodium channel. Dr. Makielski is also studying the ATP-dependent potassium channel and its role in ischemia and ischemic preconditioning in the heart. |
PubMed Articles
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Adsit GS Vaidyanathan R Galler CM Kyle JW Makielski JC .
Channelopathies from mutations in the cardiac sodium channel protein complex. J Mol Cell Cardiol. 2013 Apr 1;
[PubMed ID: 23557754]
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Cheng J Valdivia CR Vaidyanathan R Balijepalli RC Ackerman MJ Makielski JC .
Caveolin-3 suppresses late sodium current by inhibiting nNOS-dependent S-nitrosylation of SCN5A. J Mol Cell Cardiol. 2013 Mar 26;
[PubMed ID: 23541953]
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Aggarwal NT Makielski JC .
Redox control of cardiac excitability. Antioxid Redox Signal. 2013 Feb 1;18(4):432-68
[PubMed ID: 22897788]
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Mottram AR Valdivia CR Makielski JC .
Fatty acids antagonize bupivacaine-induced I(Na) blockade. Clin Toxicol (Phila). 2011 Oct;49(8):729-33
[PubMed ID: 21970771]
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Cheng J Tester DJ Tan BH Valdivia CR Kroboth S Ye B January CT Ackerman MJ Makielski JC .
The common African American polymorphism SCN5A-S1103Y interacts with mutation SCN5A-R680H to increase late Na current. Physiol Genomics. 2011 May 13;43(9):461-6
[PubMed ID: 21385947]
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Aggarwal NT Pravdic D McNally EM Bosnjak ZJ Shi NQ Makielski JC .
The mitochondrial bioenergetic phenotype for protection from cardiac ischemia in SUR2 mutant mice. Am J Physiol Heart Circ Physiol. 2010 Dec;299(6):H1884-90
[PubMed ID: 20935152]
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Cheng J Morales A Siegfried JD Li D Norton N Song J Gonzalez-Quintana J Makielski JC Hershberger RE .
SCN5A rare variants in familial dilated cardiomyopathy decrease peak sodium current depending on the common polymorphism H558R and common splice variant Q1077del. Clin Transl Sci. 2010 Dec;3(6):287-94
[PubMed ID: 21167004]
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Valdivia CR Medeiros-Domingo A Ye B Shen WK Algiers TJ Ackerman MJ Makielski JC .
Loss-of-function mutation of the SCN3B-encoded sodium channel {beta}3 subunit associated with a case of idiopathic ventricular fibrillation. Cardiovasc Res. 2010 Jun 1;86(3):392-400
[PubMed ID: 20042427]
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Tan BH Pundi KN Van Norstrand DW Valdivia CR Tester DJ Medeiros-Domingo A Makielski JC Ackerman MJ .
Sudden infant death syndrome-associated mutations in the sodium channel beta subunits. Heart Rhythm. 2010 Jun;7(6):771-8
[PubMed ID: 20226894]
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Cheng J Van Norstrand DW Medeiros-Domingo A Valdivia C Tan BH Ye B Kroboth S Vatta M Tester DJ January CT Makielski JC Ackerman MJ .
Alpha1-syntrophin mutations identified in sudden infant death syndrome cause an increase in late cardiac sodium current. Circ Arrhythm Electrophysiol. 2009 Dec;2(6):667-76
[PubMed ID: 20009079]
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Ye B Kroboth SL Pu JL Sims JJ Aggarwal NT McNally EM Makielski JC Shi NQ .
Molecular identification and functional characterization of a mitochondrial sulfonylurea receptor 2 splice variant generated by intraexonic splicing. Circ Res. 2009 Nov 20;105(11):1083-93
[PubMed ID: 19797704]
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Valdivia CR Ueda K Ackerman MJ Makielski JC .
GPD1L links redox state to cardiac excitability by PKC-dependent phosphorylation of the sodium channel SCN5A. Am J Physiol Heart Circ Physiol. 2009 Oct;297(4):H1446-52
[PubMed ID: 19666841]
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Vega AL Tester DJ Ackerman MJ Makielski JC .
Protein kinase A-dependent biophysical phenotype for V227F-KCNJ2 mutation in catecholaminergic polymorphic ventricular tachycardia. Circ Arrhythm Electrophysiol. 2009 Oct;2(5):540-7
[PubMed ID: 19843922]
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Ueda K Valdivia C 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. Proc Natl Acad Sci U S A. 2008 Jul 8;105(27):9355-60
[PubMed ID: 18591664]
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Pu JL Ye B Kroboth SL McNally EM Makielski JC Shi NQ .
Cardiac sulfonylurea receptor short form-based channels confer a glibenclamide-insensitive KATP activity. J Mol Cell Cardiol. 2008 Jan;44(1):188-200
[PubMed ID: 18001767]
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Tan BH 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. 2007 Dec 1;76(3):409-17
[PubMed ID: 17897635]
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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. 2007 Mar;4(3):323-9
[PubMed ID: 17341397]
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Tan BH Valdivia CR Song C Makielski JC .
Partial expression defect for the SCN5A missense mutation G1406R depends on splice variant background Q1077 and rescue by mexiletine. Am J Physiol Heart Circ Physiol. 2006 Oct;291(4):H1822-8
[PubMed ID: 16632547]
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Makielski JC Valdivia CR .
Ranolazine and late cardiac sodium current--a therapeutic target for angina, arrhythmia and more? Br J Pharmacol. 2006 May;148(1):4-6
[PubMed ID: 16520741]
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Makielski JC Farley AL .
Na(+) current in human ventricle: implications for sodium loading and homeostasis. J Cardiovasc Electrophysiol. 2006 May;17 Suppl 1:S15-S20
[PubMed ID: 16686671]
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Rajamani S Anderson CL Valdivia CR Eckhardt LL Foell JD Robertson GA Kamp TJ Makielski JC Anson BD January CT .
Specific serine proteases selectively damage KCNH2 (hERG1) potassium channels and I(Kr). Am J Physiol Heart Circ Physiol. 2006 Mar;290(3):H1278-88
[PubMed ID: 16227340]
