title: The contribution of the citrate pathway to oxidative stress in Down syndrome.
abstract: Inflammatory conditions and oxidative stress have a crucial role in Down syndrome (DS). Emerging studies have also reported an altered lipid profile in the early stages of DS. Our previous works demonstrate that citrate pathway activation is required for oxygen radical production during inflammation. Here, we find up-regulation of the citrate pathway and down-regulation of carnitine/acylcarnitine carrier and carnitine palmitoyl-transferase 1 genes in cells from children with DS. Interestingly, when the citrate pathway is inhibited, we observe a reduction in oxygen radicals as well as in lipid peroxidation levels. Our preliminary findings provide evidence for a citrate pathway dysregulation, which could be related to some phenotypic traits of people with DS.
authors: Department of Science, University of Basilicata, Potenza, Italy.
authors: Paolo
authors: P
authors: Convertini
authors: Department of Biosciences, Biotechnology and Pharmacological Sciences, University of Bari, Bari, Italy.
authors: Alessio
authors: A
authors: Menga
authors: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Napoli, Italy.
authors: Generoso
authors: G
authors: Andria
authors: Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Napoli, Italy.
authors: Iris
authors: I
authors: Scala
authors: Department of Science, University of Basilicata, Potenza, Italy.
authors: Anna
authors: A
authors: Santarsiero
authors: Department of Science, University of Basilicata, Potenza, Italy.
authors: Maria A
authors: MA
authors: Castiglione Morelli
authors: Department of Biosciences, Biotechnology and Pharmacological Sciences, University of Bari, Bari, Italy. vito.iacobazzi@uniba.it.
authors: Vito
authors: V
authors: Iacobazzi
authors: Department of Science, University of Basilicata, Potenza, Italy. vittoria.infantino@unibas.it.
authors: Vittoria
authors: V
authors: Infantino
mesh_terms: D027321:Anion Transport Proteins / Q000235:genetics / Q000378:metabolism*; D002331:Carnitine / Q000378:metabolism*; D002333:Carnitine Acyltransferases / Q000235:genetics / Q000378:metabolism*; D002334:Carnitine O-Palmitoyltransferase / Q000235:genetics / Q000378:metabolism*; D002461:Cell Line, Transformed; D002675:Child, Preschool; D019343:Citric Acid / Q000378:metabolism*; D004314:Down Syndrome / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D005786:Gene Expression Regulation; D006801:Humans; D007962:Leukocytes / Q000502:physiology*; D015227:Lipid Peroxidation; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism*; D027361:Organic Anion Transporters; D018384:Oxidative Stress; D010641:Phenotype; D019655:Quantitative Trait, Heritable
chemical_list: D027321:Anion Transport Proteins; D024101:Mitochondrial Proteins; D027361:Organic Anion Transporters; C532592:Slc25a1 protein, human; D019343:Citric Acid; D002333:Carnitine Acyltransferases; D002334:Carnitine O-Palmitoyltransferase; D002331:Carnitine
keywords: ATP citrate lyase; Down syndrome; citrate pathway; inflammation; mitochondrial citrate carrier; oxidative stress