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Arnold W. Strauss, MDBK Rachford Professor and Chair, Department of Pediatrics Director, Cincinnati Children's Research Foundation
Dr. Strauss’ laboratory focuses on energy generation by mitochondria through fatty acid oxidation (FAO). Highly oxidative tissues such as liver, heart, skeletal muscle, gut, and kidney rely upon FAO for energy. Intermediary metabolism of fatty acids in the liver is the major source of short chain fatty acids (“ketone bodies”) that are important fuels in the brain. The fetal-maternal metabolic transition is a switch from glucose to fatty acids as the major energy source. Dr. Strauss has cloned and characterized mouse and human genes encoding five enzymes of the FAO pathway-medium chain acyl-CoA dehydrogenase (MCAD), very long chain acyl-CoA dehydrogenase (VLCAD), the two genes of the trifunctional protein (TFP) subunits, and medium and short chain 3-hydroxy-acyl-CoA dehydrogenase (SCHAD). Dr. Strauss is investigating the molecular genetics of infants and children with mutations in these genes causing hypoketotic hypoglycemia and acute liver failure. Additionally, Dr. Strauss is defining the relationship between fetal FAO defects and the development of severe liver diseases during pregnancy, including acute fatty liver of pregnancy and the HELLP syndrome.
Dr. Strauss collaborates with Dr. Bucuvalas on the U01 grant investigating acute liver failure in children. Projection of Core use: Biostatistical services
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Mechanism of insulin dysregulation in SCHAD deficiency. In SCHAD deficient states, the loss of an inhibitory protein-protein interaction of SCHAD on GDH leads to an increase in the sensitivity of the GDH reaction to allosteric activation by leucine. Stimulation of cells by glutamine plus leucine produces a submaximal insulin response compared to stimulation with a complete physiologic mixture of amino acids. Figure from J Biol Chem, 2010; 285:31806-31818.
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