Title :

Fetal bovine serum impacts the observed N‐glycosylation defects in TMEM165 KO HEK cells

Author(s) :

Vicogne, Dorothee [Auteur]
Unité de Glycobiologie Structurale et Fonctionnelle UMR 8576 [UGSF]
Houdou, Marine [Auteur]
Unité de Glycobiologie Structurale et Fonctionnelle - UMR 8576 [UGSF]
Garat, Anne [Auteur]
Impact de l'environnement chimique sur la santé humaine - ULR 4483 [IMPECS]
Centre Hospitalier Régional Universitaire [Lille] [CHRU Lille]
Climer, Leslie [Auteur]
Baylor University
Lupashin, Vladimir [Auteur]
University of Arkansas for Medical Sciences [UAMS]
Morelle, Willy [Auteur]
Unité de Glycobiologie Structurale et Fonctionnelle UMR 8576 [UGSF]
Foulquier, Francois [Auteur]
Unité de Glycobiologie Structurale et Fonctionnelle UMR 8576 [UGSF]

Journal title :

Journal of Inherited Metabolic Disease

Abbreviated title :

Jrnl of Inher Metab Disea

Volume number :

43

Pages :

357-366

Publisher :

Wiley

Publication date :

2019-10-01

English keyword(s) :

FBS
manganese level
N‐glycosylation defects
TMEM165

HAL domain(s) :

Sciences du Vivant [q-bio]
Chimie/Chimie théorique et/ou physique

English abstract : [en]

TMEM165 is involved in a rare genetic human disease named TMEM165‐CDG (congenital disorders of glycosylation). It is Golgi localized, highly conserved through evolution and belongs to the uncharacterized protein family ...
Show more >TMEM165 is involved in a rare genetic human disease named TMEM165‐CDG (congenital disorders of glycosylation). It is Golgi localized, highly conserved through evolution and belongs to the uncharacterized protein family 0016 (UPF0016). The use of isogenic TMEM165 KO HEK cells was crucial in deciphering the function of TMEM165 in Golgi manganese homeostasis. Manganese is a major cofactor of many glycosylation enzymes. Severe Golgi glycosylation defects are observed in TMEM165 Knock Out Human Embryonic Kidney (KO HEK) cells and are rescued by exogenous manganese supplementation. Intriguingly, we demonstrate in this study that the observed Golgi glycosylation defect mainly depends on fetal bovine serum, particularly its manganese level. Our results also demonstrate that iron and/or galactose can modulate the observed glycosylation defects in TMEM165 KO HEK cells. While isogenic cultured cells are widely used to study the impact of gene defects on proteins' glycosylation patterns, these results emphasize the importance of the use of validated fetal bovine serum in glycomics studies.Show less >

Language :

Anglais

Peer reviewed article :

Oui

Audience :

Internationale

Popular science :

Non

European Project :

ERA-NET rare disease research implementing IRDiRC objectives

ANR Project :

Décryptage des patients CDG (Congenital Disorders of Glyvosylation) déficients en TMEM165 - de la compréhension des mécanismes moléculaires à une thérapie
A European research network directed towards improving diagnosis and treatment of inborn glycosylation disorders.

Administrative institution(s) :

Université de Lille
CNRS

Collections :

• IMPact de l'Environnement Chimique sur la Santé humaine (IMPECS) - ULR 4483
• Unité de Glycobiologie Structurale et Fonctionnelle (UGSF) - UMR 8576

Research team(s) :

Mécanismes moléculaires de la N-glycosylation et pathologies associées

Submission date :

2020-12-18T13:44:24Z
2021-01-04T10:15:15Z
2021-01-05T11:25:14Z
2023-07-07T11:27:03Z
2023-07-07T11:46:55Z