Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10461663 | Neuroscience & Biobehavioral Reviews | 2014 | 16 Pages |
Abstract
We first report latest advances on genetic and environmental regulation of mitochondrial function and its role in brain development. Starting from the structure, function and regulation of the oxidative phosphorylation apparatus, we review how mitochondrial biogenesis and dynamics play a central role in neurogenesis and neuroplasticity. We then discuss how dysfunctional mitochondria and alterations in reactive oxygen species homeostasis are potentially involved in the pathogenesis of various neurodevelopmental syndromes with a special focus on Down, Rett, Fragile X syndromes and autism spectrum disorders. Finally, we review and suggest novel therapeutic approaches aimed at improving intellectual disability by activating mitochondrial function and reducing oxidative stress to amiliorate the quality of life in the subjects affected.
Keywords
NRF1MFN2ANTEpigallocatechin-3-gallateRNSRTTMRCMfn1pKaCREBIPSCsDYRK1ATFAMMecp2FMRPSOD1Drp1FMROXPHOSEGCGDSCR1fragile X mental retardationfragile X-associated tremor/ataxia syndromePGC1αFSP1cAMP responsive element-binding proteinHsa213′-5′-cyclic adenosine monophosphatecAMPFXTASROSMitochondrial dysfunctionautism spectrum disordersAutismMitochondrial diseasesOxidative stressDrug developmentadenine nucleotide transporterCNSMitochondrial respiratory chainInduced pluripotent stem cellsDown syndromeRett syndromesuperoxide dismutase 1central nervous systemfragile X Nuclear respiratory factor 1Mitochondrial transcription factorOxidative phosphorylationmitofusin 2MitochondriaMicroRNAMiRNAintellectual disabilityNADHASDNeuroplasticityNeurogenesisfragile X mental retardation proteinmethyl-CpG-binding protein 2dynamin-related protein 1protein kinase Areduced nicotinamide adenine dinucleotideHuman chromosome 21reactive nitrogen speciesReactive oxygen species
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Authors
Daniela Valenti, Lidia de Bari, Bianca De Filippis, Alexandra Henrion-Caude, Rosa Anna Vacca,