Titre :

GlcNAc6ST-1 regulates sulfation of N-glycans and myelination in the peripheral nervous system

Auteur(s) :

Yoshimura, Takeshi [Auteur]
Hayashi, Akiko [Auteur]
Handa-Narumi, Mai [Auteur]
Yagi, Hirokazu [Auteur]
Ohno, Nobuhiko [Auteur]
Koike, Takako [Auteur]
Yamaguchi, Yoshihide [Auteur]
Uchimura, Kenji [Auteur]
Unité de Glycobiologie Structurale et Fonctionnelle - UMR 8576 [UGSF]
Kadomatsu, Kenji [Auteur]
Sedzik, Jan [Auteur]
Kitamura, Kunio [Auteur]
Kato, Koichi [Auteur]
Trapp, Bruce D. [Auteur]
Baba, Hiroko [Auteur]
Ikenaka, Kazuhiro [Auteur]

Titre de la revue :

Scientific Reports

Nom court de la revue :

Sci Rep

Numéro :

7

Pagination :

42257

Date de publication :

2017-02-10

ISSN :

2045-2322

Discipline(s) HAL :

Chimie/Chimie théorique et/ou physique

Résumé en anglais : [en]

Highly specialized glial cells wrap axons with a multilayered myelin membrane in vertebrates. Myelin serves essential roles in the functioning of the nervous system. Axonal degeneration is the major cause of permanent ...
Lire la suite >Highly specialized glial cells wrap axons with a multilayered myelin membrane in vertebrates. Myelin serves essential roles in the functioning of the nervous system. Axonal degeneration is the major cause of permanent neurological disability in primary myelin diseases. Many glycoproteins have been identified in myelin, and a lack of one myelin glycoprotein results in abnormal myelin structures in many cases. However, the roles of glycans on myelin glycoproteins remain poorly understood. Here, we report that sulfated N-glycans are involved in peripheral nervous system (PNS) myelination. PNS myelin glycoproteins contain highly abundant sulfated N-glycans. Major sulfated N-glycans were identified in both porcine and mouse PNS myelin, demonstrating that the 6-O-sulfation of N-acetylglucosamine (GlcNAc-6-O-sulfation) is highly conserved in PNS myelin between these species. P0 protein, the most abundant glycoprotein in PNS myelin and mutations in which at the glycosylation site cause Charcot-Marie-Tooth neuropathy, has abundant GlcNAc-6-O-sulfated N-glycans. Mice deficient in N-acetylglucosamine-6-O-sulfotransferase-1 (GlcNAc6ST-1) failed to synthesize sulfated N-glycans and exhibited abnormal myelination and axonal degeneration in the PNS. Taken together, this study demonstrates that GlcNAc6ST-1 modulates PNS myelination and myelinated axonal survival through the GlcNAc-6-O-sulfation of N-glycans on glycoproteins. These findings may provide novel insights into the pathogenesis of peripheral neuropathy.Lire moins >

Langue :

Anglais

Établissement(s) :

CNRS
Université de Lille

Collections :

• Unité de Glycobiologie Structurale et Fonctionnelle (UGSF) - UMR 8576

Date de dépôt :

2020-02-12T15:11:39Z