• Dystrophic Cardiomyopathy
  • Role of Dystrophin
  • Role of Hypoxia
  • Hemodynamics

Dystrophic Cardiomyopathy Pathogenesis

An important goal of our laboratory is understanding the basic mechanisms of the heart disease associated with muscular dystrophy. Heart disease is already the second leading cause of death in Duchenne muscular dystrophy patients and its importance is increasing with improvements in respiratory care. Unfortunately, we know very little about treating dystrophic cardiomyopathy - our lab is working to change this.

Dystrophic Lesion

Dystrophin Function

A significant goal of our laboratory is to understand the function of dystrophin in the heart. Our studies suggest that the assembly of dystrophin with its glycoprotein partners may occur early in the secretory pathways suggesting a possibility that dystrophin has a role in getting components of the dystrophin glycoprotein complex to the membrane. These studies use a variety of biochemistyr and genetic modifications.

DGC Schematic

Hypoxia Reveals Cardiac Dysfunction

Recent work in mice has demonstrated that decreased oxygen (hypoxia) results in significant cardiac damage in dystrophic hearts. We are continuing to explore this observation, branching into metabolomics and mitochondrial function.

Hypoxic Hearts

In Vivo Hemodynamics

Our laboratory takes an integrative approach to studying heart disease. This includes the use of invasive left ventricular catheterization, non-invasive echocardiography, and studies of isolated hearts, vessels, and cardiac myocytes. These approaches provide unique perspectives on cardiac physiology and pathophysiology.

PV Loops