Pitt Pediatrics congratulates Bianca Seminotti, MSc, PhD, for her Best Poster Award from the INFORM Program (International Network of Fatty Acid Oxidation Research and Management). Her research was titled “Restoring succinyllysine antigenic signal and improving O2 consumption of CPT II deficient cells treated with anaplerotic compounds.”
Seminotti’s research is focused on developing a more effective treatment to remedy the deficiency in energy intermediates caused by long and medium chain fatty acid oxidation disorders, including carnitine palmitoyltransferase II (CPTII) deficiency. This deficiency is an autosomal recessive disorder that restricts long-chain FA entry into mitochondria causing energy and other dysfunctions. Medium branched-chain fatty acids have shown improvement in metabolite profiles suggesting possible advantage, as triglycerides, to treat FAODs as they provide two propionyl-CoAs instead of one as is generated from C7.
Seminotti currently works as a Research Scientist in the Vockley Lab.