Abnormal N-glycosylation pattern for brain nucleotide pyrophosphatase-5 (NPP-5) in Mecp2-mutant murine models of Rett syndrome - Archive ouverte HAL Accéder directement au contenu
Article Dans Une Revue Neuroscience Research Année : 2016

Abnormal N-glycosylation pattern for brain nucleotide pyrophosphatase-5 (NPP-5) in Mecp2-mutant murine models of Rett syndrome

Claudio de Felice
Roberto Guerranti
  • Fonction : Auteur
Cinzia Signorini
Silvia Leoncini
Alessandra Pecorelli
  • Fonction : Auteur
Francesco Scalabrì
  • Fonction : Auteur
Cinzia Della Giovampaola
  • Fonction : Auteur
Antonietta Capone
  • Fonction : Auteur
Cristiana Mirasole
  • Fonction : Auteur
Lello Zolla
  • Fonction : Auteur
Giuseppe Valacchi
  • Fonction : Auteur
Lucia Ciccoli
  • Fonction : Auteur
Joussef Hayek
  • Fonction : Auteur
Maurizio D’esposito
  • Fonction : Auteur

Résumé

Neurological disorders can be associated with protein glycosylation abnormalities. Rett syndrome is a devastating genetic brain disorder, mainly caused by de novo loss-of-function mutations in the methyl-CpG binding protein 2 (MECP2) gene. Although its pathogenesis appears to be closely associated with a redox imbalance, no information on glycosylation is available. Glycoprotein detection strategies (i.e., lectin-blotting) were applied to identify target glycosylation changes in the whole brain of Mecp2 mutant murine models of the disease. Remarkable glycosylation pattern changes for a peculiar 50kDa protein, i.e., the N-linked brain nucleotide pyrophosphatase-5 were evidenced, with decreased N-glycosylation in the presymptomatic and symptomatic mutant mice. Glycosylation changes were rescued by selected brain Mecp2 reactivation. Our findings indicate that there is a causal link between the amount of Mecp2 and the N-glycosylation of NPP-5.
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hal-03576837 , version 1 (16-02-2022)

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Alessio Cortelazzo, Claudio de Felice, Roberto Guerranti, Cinzia Signorini, Silvia Leoncini, et al.. Abnormal N-glycosylation pattern for brain nucleotide pyrophosphatase-5 (NPP-5) in Mecp2-mutant murine models of Rett syndrome. Neuroscience Research, 2016, 105, pp.28-34. ⟨10.1016/j.neures.2015.10.002⟩. ⟨hal-03576837⟩
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