Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: An overview of Down syndrome, autism, Fragile X and Rett syndrome (Articolo in rivista)

Type

• Prodotto della ricerca (Classe)
• Articolo in rivista (Classe)

Label

• Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: An overview of Down syndrome, autism, Fragile X and Rett syndrome (Articolo in rivista) (literal)

Anno

• 2014-01-01T00:00:00+01:00 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi

• 10.1016/j.neubiorev.2014.01.012 (literal)

Alternative label

• Valenti D.; de Bari L.; De Filippis B.; Henrion-Caude A.; Vacca R.A. (2014)
  Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: An overview of Down syndrome, autism, Fragile X and Rett syndrome
  in Neuroscience and biobehavioral reviews; Elsevier, New York (Stati Uniti d'America)
  (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori

• Valenti D.; de Bari L.; De Filippis B.; Henrion-Caude A.; Vacca R.A. (literal)

Pagina inizio

• 202 (literal)

Pagina fine

• 217 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#url

• http://www.scopus.com/inward/record.url?eid=2-s2.0-84895096331&partnerID=q2rCbXpz (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume

• 46 (literal)

Rivista

• Neuroscience and biobehavioral reviews (Rivista)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#pagineTotali

• 16 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroFascicolo

• 2 (literal)

Note

• ISI Web of Science (WOS) (literal)
• Scopu (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni

• Institute of Biomembranes and Bioenergetics, National Council of Research, Bari, Italy; Section of Behavioural Neuroscience, Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità Roma, Italy; INSERM U781 Hôpital Necker - Enfants Malades, Paris, France (literal)

Titolo

• Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: An overview of Down syndrome, autism, Fragile X and Rett syndrome (literal)

Abstract

• Clinical manifestations typical of mitochondrial diseases are often present in various genetic syndromes associated with intellectual disability, a condition leading to deficit in cognitive functions and adaptive behaviors. Until now, the causative mechanism leading to intellectual disability is unknown and the progression of the condition is poorly understood. 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. © 2014 Elsevier Ltd. All rights reserved. (literal)

Editore

• Elsevier (Editore)

Prodotto di

• Interrelazione nucleo/citoplasma/mitocondri nell'omeostasi cellulare. (SV.P15.003.001) (Modulo)
• Istituto di biomembrane e bioenergetica (IBBE) (Istituto)

Autore CNR

• DANIELA VALENTI (Persona)
• LIDIA DE BARI (Unità di personale interno)
• ROSA ANNA VACCA (Persona)

Insieme di parole chiave

• Parole chiave di "Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: An overview of Down syndrome, autism, Fragile X and Rett syndrome" (Insieme di parole chiave)

Incoming links:

Prodotto

• Istituto di biomembrane e bioenergetica (IBBE) (Istituto)
• Interrelazione nucleo/citoplasma/mitocondri nell'omeostasi cellulare. (SV.P15.003.001) (Modulo)

Autore CNR di

• ROSA ANNA VACCA (Persona)
• DANIELA VALENTI (Persona)
• LIDIA DE BARI (Unità di personale interno)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#rivistaDi

• Neuroscience and biobehavioral reviews (Rivista)

Editore di

• Elsevier (Editore)

Insieme di parole chiave di

• Parole chiave di "Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: An overview of Down syndrome, autism, Fragile X and Rett syndrome" (Insieme di parole chiave)