Timothy Kamp
University of Wisconsin-Madison, WI·Physiology
Some inherited heart conditions cause dangerous, sometimes fatal irregular heartbeats that start in one specific region of the heart. Brugada syndrome is one of them: clinical studies point to the outer surface of the right ventricular outflow tract, the channel through which the right side of the heart pumps blood toward the lungs, as the place where these rhythms begin.
- Fields
Physiology
Medicine & Clinical Research
Biomedical Engineering
- Topics
Brugada syndrome
cardiac arrhythmia
sudden cardiac death
- Grants
- 1 known grant, through Apr 2030
What they work on
- Test whether low oxygen drives second heart field progenitors toward right ventricular and outflow tract cardiomyocytes, and whether Brugada syndrome patient cells show developmental defects such as hypoxia-induced apoptosis.
- Build an AI assistant (SteMy) to improve the reproducibility of stem cell differentiation protocols for right ventricular outflow tract cardiomyocytes.
- Determine whether Brugada syndrome cardiac tissue chips containing patient outflow tract and epicardial cells show reduced, heterogeneous conduction, using optical mapping, cellular electrophysiology, immunolabeling and single-cell RNA sequencing.
- Discriminate the mechanisms of Brugada syndrome arising from SCN5A missense mutations, nonsense mutations and genotype-negative cases, testing for reduced sodium current, increased transient outward current and Nav1.5/Cx43 mislocalization.
- Quantify how far anticodon-engineered suppressor tRNAs restore Nav1.5 expression, sodium current and conduction velocity across a range of premature stop codons in patient-derived cells, and measure off-target effects.
More about the research
Decades of work have produced no preventive treatment, partly because some patients carry mutations in SCN5A, the gene for the heart's main sodium channel, while in most patients the cause is unknown, and partly because there has been no good human laboratory model of this particular region of the heart.
Why it matters
Brugada syndrome causes sudden cardiac death and currently has no preventive therapy. Human stem cell models of the right ventricular outflow tract, where its arrhythmias arise, could clarify why rhythms become unstable in patients with and without SCN5A mutations. Engineered tRNAs that read through premature stop mutations could become a first disease-modifying treatment for a subset of patients, and the same approach may apply to other genetic conditions caused by nonsense mutations.
Known grants
From NIH and NSF research grants. Labs often have other funding we can't see.
Closest labs
Manu Ben Johny
Columbia University, NY·Biophysics
Determine whether CaV2.1 and CaV2.2 calcium channels are S-palmitoylated, carrying an attached lipid group, in central and peripheral neurons, and how this modification alters channel activity.
Robert Piper
University of Iowa, IA·Physiology
Produce stably expressing human HEK cell lines that pair individual β subunits (Scn2b, Scn3b, Scn4b) with specific pore-forming NaV α subunits.
Christopher A Ahern
University of Iowa, IA·Biophysics
Develop light-activated photochemical 'stapling' to lock voltage-gated sodium, calcium and potassium channels into key shapes in live cell membranes before purification for structural determination.
The school
University of Wisconsin-Madison: 91 labs on PI Pages, $1.9B a year on research, 854 research doctorates a year.
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