Resveratrol-Induced Changes in MicroRNA Expression in Primary Human Fibroblasts Harboring Carnitine-Palmitoyl Transferase-2 Gene Mutation, Leading to Fatty Acid Oxidation Deficiency.
Virginie AiresDominique DelmasFatima DjouadiJean BastinMustapha Cherkaoui-MalkiNorbert LatruffePublished in: Molecules (Basel, Switzerland) (2017)
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.
Keyphrases
- end stage renal disease
- fatty acid
- poor prognosis
- ejection fraction
- chronic kidney disease
- newly diagnosed
- prognostic factors
- cell proliferation
- hydrogen peroxide
- respiratory syncytial virus
- nitric oxide
- long non coding rna
- binding protein
- replacement therapy
- signaling pathway
- late onset
- inflammatory response
- pi k akt
- combination therapy
- toll like receptor
- induced pluripotent stem cells
- reactive oxygen species
- duchenne muscular dystrophy