About Zhuqing’s research:
My project focuses on understanding the fundamental molecular mechanism of Barth syndrome pathology. Barth syndrome is a rare genetic disease characterized by cardiomyopathy, skeletal myopathy, neutropenia, and a host of metabolic irregularities. As such, Barth syndrome patients have a limited capacity for exercising and experience fatigue even when performing common everyday activities. Among the metabolic features associated with Barth syndrome, pyruvate dehydrogenase (PDH) deficiency represents a likely factor contributing to energy insufficiency and exercise intolerance. PDH is a central metabolic node that mediates oxidation of glycolysis-derived pyruvate to acetyl-CoA, fueling the citric acid (TCA) cycle. The activity of PDH is highly dependent on reversible phosphorylation by PDH kinases and dephosphorylation by PDH phosphatases. My studies show that these two types of regulatory proteins play important roles in inactivating PDH in the context of Barth syndrome.
My other research focus is to characterize and determine the contribution of defects in synthesis and maturation of iron-sulfur (Fe-S) clusters in Barth syndrome. Fe-S clusters are conserved cofactors that bind to many proteins, including electron transport chain and TCA cycle components, and regulate their activity. There are many intriguing questions and exciting findings to be discovered on this project!
I implement multiple research models to address my research questions, including C2C12 myoblasts, yeast, and mouse tissues. I also have experience working with whole-animal mouse models.