Children's Hospital is part of the UPMC family.
Our Sites
Be safe anytime, anywhere.
To find a pediatrician or pediatric specialist, please call 412-692-7337 or search our directory.
A resource for our network of referring physicians.
For more information about research, please call our main office at 412-692-6438.
Ranked #8 Nationally by U.S. News & World Report.
The regulation of fatty acid oxidation and its implications for diseases such as diabetes and obesity has been a key focus of Eric Goetzman, PhD.
Otto Warburg observed in 1924 that cancer cells have an unusual reliance on glycolysis for energy. He believed this metabolic switch to be indicative of a defect in mitochondrial oxidative metabolism. More than 90 years later, researchers still do not understand how and why cancer cells alter their metabolism or what role changes in mitochondrial energy metabolism play in the etiology of cancer or in the ability of cancer cells to escape apoptosis. Answering these questions may reveal cancer’s Achilles’ heel and lead to a cure. Our team is collaborating with Edward V. Prochownik, MD, PhD, director of Oncology Research at UPMC Children’s Hospital of Pittsburgh, to study how the oncogenic transcription factor c-Myc drives cells toward an anabolic metabolic phenotype. This may involve changes in mitochondrial enzyme lysine acetylation as well as changes in partitioning between glucose, glutamine, and fatty acid metabolism.
Eric Goetzman, PhD
In mouse models, those “knocked out” for the fatty acid oxidation (FAO) enzyme LCAD demonstrate reduced lung function. Our research team hypothesizes that LCAD and the FAO pathway are involved in synthesizing and secreting pulmonary surfactant in a specialized lung cell known as the type II pneumocyte. Surfactant is a mixture of phospholipids and proteins that reduces surface tension in the lung. This effect is necessary to prevent the collapse of the alveoli and promote gas exchange. LCAD knockout mice have reduced amounts of surfactant lipids and an altered phospholipid composition. Preliminary studies show an increased susceptibility to infection by influenza. Efforts are underway to determine the molecular mechanisms behind these changes.
A core focus of our laboratory studies is mitochondrial fatty acid oxidation (FAO), the pathway by which fatty acids are broken down for energy. Mutations in the FAO genes are among the most prevalent inborn errors of metabolism. Recently, it was discovered that the FAO enzymes are heavily modified by post-translational modifications, including lysine acetylation and lysine succinylation. There are three mitochondrial sirtuin deacylases (SIRT3, SIRT4, SIRT5), which are believed to reverse some of these modifications. The lab’s current research focuses on the functional effects of lysine acetylation and succinylation on the FAO pathway and the role the sirtuins play in regulating metabolism.
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
UPMC Children’s Hospital of Pittsburgh One Children’s Hospital Way 4401 Penn Ave. Pittsburgh, PA 15224
In addition to the main hospital, Children's has many convenient locations in other neighborhoods throughout the greater Pittsburgh region.
With MyCHP, you can request appointments, review test results, and more.
For questions about a hospital bill call:
To pay your bill online, please visit UPMC's online bill payment system.
Interested in giving to Children's Hospital? Support the hospital by making a donation online, joining our Heroes in Healing monthly donor program, or visiting our site to learn about the other ways you can give back.