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Prof. Dr.
Ute Spiekerkötter

Department of General Pediatrics, Adolescent Medicine and Neonatology
Medical Director


  • 1995-2001 Center for Pediatrics, University Hospital Düsseldorf, Germany, Specialist training in Pediatrics
  • 2001-2003 Vanderbilt University, Nashville, TN, USA, Postdoctoral Fellowship
  • 2004-2005 University of Amsterdam, The Netherlands, Casimir-Ziegler Research Stipend, Postdoctoral Fellowship
  • 2005-2012 Center for Pediatrics, University Hospital Düsseldorf, Specialist training in pediatric endocrinology and diabetes, Consultant
  • 2008-2012 Professor (W2) of Inborn errors of Metabolism
  • Since 2012 Full Professor (W3) and Medical Director, Department of General Pediatrics, Adolescent medicine and Neonatology, Medical Center – University of Freiburg, Germany

Focus of research

  • Inborn errors of fatty acid oxidation: pathophysiology and new therapies
  • Mitochondrial morphology and function in genetic enzyme defects of energy metabolism
  • Learning in an interprofessional context, training ward for medical students and nursing students (IPA Päd)

Selected publications

  • Tucci S, ... Spiekerkoetter U. Triheptanoin: long-term effects in the very long-chain acyl-CoA dehydrogenase (VLCAD-/-)-deficient mouse. J Lipid Res. 2017; 58(1):196-207.
  • Tucci S, Behringer S, Spiekerkoetter U. De novo fatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even mediumchain fatty acids. FEBS J. 2015; 282(21):4242-53.
  • Tucci S, Flögel U, Spiekerkoetter U. Sexual dimorphism of lipid metabolism in very long-chain acyl-CoA dehydrogenase deficient (VLCAD-/-) mice in response to medium-chain triglycerides (MCT). Biochim Biophys Acta. 2015; 1852(7):1442-50.
  • Koster KL, ... Spiekerkoetter U. Functional studies of 18 heterologously expressed medium-chain acyl-CoA dehydrogenase (MCAD) variants. J Inherit Metab Dis. 2014; 37(6):917-28.
  • Tucci S, ... Spiekerkoetter U. Development and pathomechanisms of cardiomyopathy in very long-chain acyl-CoA dehydrogenase deficient (VLCAD(-/-)) mice. Biochim Biophys Acta. 2014; 1842(5):677-85.

Research methods

  • Metabolic profiling / tandem mass spectrometry
  • Functional characterization of proteins and enzymes
  • Genetic characterization of rare inborn errors of metabolism / Panel diagnostics, WES