Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency
Carnitine palmitoyltransferase-2 (CPT2) is a mitochondrial enzyme involved in long-chain fatty acid entry into mitochondria for their β-oxidation and energy production. Two phenotypes are associated with the extremely reduced CPT2 activity in genetically deficient patients: neonatal lethality or, in...
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doaj-9339ad9072d14a31b1be550b01d1ebde2020-11-25T01:31:50ZengMDPI AGMolecules1420-30492017-12-01231710.3390/molecules23010007molecules23010007Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation DeficiencyVirginie Aires0Dominique Delmas1Fatima Djouadi2Jean Bastin3Mustapha Cherkaoui-Malki4Norbert Latruffe5Laboratory BioPeroxIL, University of Bourgogne-Franche Comté, 6 Blvd Gabriel, 21000 Dijon, FranceLaboratory BioPeroxIL, University of Bourgogne-Franche Comté, 6 Blvd Gabriel, 21000 Dijon, FranceINSERM U1124, Université Paris-Descartes, Rue des Saints-Pères, 75000 Paris, FranceINSERM U1124, Université Paris-Descartes, Rue des Saints-Pères, 75000 Paris, FranceLaboratory BioPeroxIL, University of Bourgogne-Franche Comté, 6 Blvd Gabriel, 21000 Dijon, FranceLaboratory BioPeroxIL, University of Bourgogne-Franche Comté, 6 Blvd Gabriel, 21000 Dijon, FranceCarnitine palmitoyltransferase-2 (CPT2) is a mitochondrial enzyme involved in long-chain fatty acid entry into mitochondria for their β-oxidation and energy production. Two phenotypes are associated with the extremely reduced CPT2 activity in genetically deficient patients: neonatal lethality or, in milder forms, myopathy. Resveratrol (RSV) is a phytophenol produced by grape plant in response to biotic or abiotic stresses that displays anti-oxidant properties, in particular through AP-1, NFκB, STAT-3, and COX pathways. Some beneficiary effects of RSV are due to its modulation of microRNA (miRNA) expression. RSV can enhance residual CPT2 activities in human fibroblasts derived from CPT2-deficient patients and restores normal fatty acid oxidation rates likely through stimulation of mitochondrial biogenesis. Here, we report changes in miRNA expression linked to CPT2-deficiency, and we identify miRNAs whose expression changed following RSV treatment of control or CPT2-deficient fibroblasts isolated from patients. Our findings suggest that RSV consumption might exert beneficiary effects in patients with CPT2-deficiency.https://www.mdpi.com/1420-3049/23/1/7resveratrolmiRNA levelCPT2-deficient cells |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Virginie Aires Dominique Delmas Fatima Djouadi Jean Bastin Mustapha Cherkaoui-Malki Norbert Latruffe |
spellingShingle |
Virginie Aires Dominique Delmas Fatima Djouadi Jean Bastin Mustapha Cherkaoui-Malki Norbert Latruffe Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency Molecules resveratrol miRNA level CPT2-deficient cells |
author_facet |
Virginie Aires Dominique Delmas Fatima Djouadi Jean Bastin Mustapha Cherkaoui-Malki Norbert Latruffe |
author_sort |
Virginie Aires |
title |
Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency |
title_short |
Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency |
title_full |
Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency |
title_fullStr |
Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency |
title_full_unstemmed |
Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency |
title_sort |
resveratrol-induced changes in microrna expression in primary human fibroblasts harboring carnitine-palmitoyl transferase-2 gene mutation, leading to fatty acid oxidation deficiency |
publisher |
MDPI AG |
series |
Molecules |
issn |
1420-3049 |
publishDate |
2017-12-01 |
description |
Carnitine palmitoyltransferase-2 (CPT2) is a mitochondrial enzyme involved in long-chain fatty acid entry into mitochondria for their β-oxidation and energy production. Two phenotypes are associated with the extremely reduced CPT2 activity in genetically deficient patients: neonatal lethality or, in milder forms, myopathy. Resveratrol (RSV) is a phytophenol produced by grape plant in response to biotic or abiotic stresses that displays anti-oxidant properties, in particular through AP-1, NFκB, STAT-3, and COX pathways. Some beneficiary effects of RSV are due to its modulation of microRNA (miRNA) expression. RSV can enhance residual CPT2 activities in human fibroblasts derived from CPT2-deficient patients and restores normal fatty acid oxidation rates likely through stimulation of mitochondrial biogenesis. Here, we report changes in miRNA expression linked to CPT2-deficiency, and we identify miRNAs whose expression changed following RSV treatment of control or CPT2-deficient fibroblasts isolated from patients. Our findings suggest that RSV consumption might exert beneficiary effects in patients with CPT2-deficiency. |
topic |
resveratrol miRNA level CPT2-deficient cells |
url |
https://www.mdpi.com/1420-3049/23/1/7 |
work_keys_str_mv |
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