Accéder directement au contenu Accéder directement à la navigation
Nouvelle interface
Article dans une revue

Mitochondrial dysfunction in CA1 hippocampal neurons of the UBE3A deficient mouse model for Angelman syndrome

Abstract :

Angelman syndrome (AS) is a severe neurological disorder caused by a deficiency of ubiquitin protein ligase E3A (UBE3A), but the pathophysiology of the disease remains unknown. We now report that in the brains of AS mice in which the maternal UBE3A allele is mutated (m-) and the paternal allele is potentially inactivated by imprinting (p+) (UBE3A m-\p+), the mitochondria are abnormal and exhibit a partial oxidative phosphorylation (OXPHOS) defect. Electron microscopy of the hippocampal region of the UBE3A m-\p+ mice (n=6) reveals small, dense mitochondria with altered cristae, relative to wild-type littermates (n=6) and reduced synaptic vesicle density. The specific activity of OXPHOS complex III is reduced in whole brain mitochondria in UBE3A m-\p+ (n=5) mice versus wild-type littermates (n=5). Therefore, mitochondrial dysfunction may contribute to the pathophysiology of Angelman syndrome.

Type de document :
Article dans une revue
Liste complète des métadonnées

https://hal.univ-angers.fr/hal-03408480
Contributeur : Okina Univ Angers Connectez-vous pour contacter le contributeur
Soumis le : vendredi 29 octobre 2021 - 10:45:19
Dernière modification le : samedi 30 octobre 2021 - 03:57:58

Lien texte intégral

Identifiants

Collections

Citation

H. Su, W. Fan, P. Coskun, J. Vesa, J. Gold, et al.. Mitochondrial dysfunction in CA1 hippocampal neurons of the UBE3A deficient mouse model for Angelman syndrome. Neuroscience Letters, 2011, 487 (2), pp.129 - 33. ⟨10.1016/j.neulet.2009.06.079⟩. ⟨hal-03408480⟩

Partager

Métriques

Consultations de la notice

4