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.

The contribution of the citrate pathway to oxidative stress in Down syndrome

Menga A.;
2016-01-01

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.
2016
149
4
423
431
ATP citrate lyase; Down syndrome; citrate pathway; inflammation; mitochondrial citrate carrier; oxidative stress; Anion Transport Proteins; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Cell Line, Transformed; Child, Preschool; Citric Acid; Down Syndrome; Gene Expression Regulation; Humans; Leukocytes; Lipid Peroxidation; Mitochondrial Proteins; Organic Anion Transporters; Oxidative Stress; Phenotype; Quantitative Trait, Heritable
Convertini P.; Menga A.; Andria G.; Scala I.; Santarsiero A.; Castiglione Morelli M.A.; Iacobazzi V.; Infantino V.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/1869093
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