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Fetal bovine serum impacts the observed N‐glycosylation defects in TMEM165 KO HEK cells
- Source :
- Journal of Inherited Metabolic Disease, Journal of Inherited Metabolic Disease, 2019, ⟨10.1002/jimd.12161⟩, Journal of Inherited Metabolic Disease, Springer Verlag, 2019, ⟨10.1002/jimd.12161⟩, Journal of Inherited Metabolic Disease, Springer Verlag, 2019, 214 (12), pp.201817815. ⟨10.1002/jimd.12161⟩
- Publication Year :
- 2019
- Publisher :
- HAL CCSD, 2019.
-
Abstract
- 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. 43;2
- Subjects :
- Glycosylation
Protein family
[SDV]Life Sciences [q-bio]
Golgi Apparatus
FBS
manganese level
N‐glycosylation defects
TMEM165
Article
Antiporters
Glycomics
03 medical and health sciences
chemistry.chemical_compound
symbols.namesake
Congenital Disorders of Glycosylation
0302 clinical medicine
N-linked glycosylation
Genetics
Humans
[SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology
[SDV.BBM.BC]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biochemistry [q-bio.BM]
Cation Transport Proteins
Genetics (clinical)
ComputingMilieux_MISCELLANEOUS
030304 developmental biology
Manganese
0303 health sciences
Ion Transport
HEK 293 cells
Serum Albumin, Bovine
Golgi apparatus
Embryonic stem cell
Cell biology
[SDV.BBM.BC]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biomolecules [q-bio.BM]
carbohydrates (lipids)
HEK293 Cells
chemistry
symbols
Calcium
030217 neurology & neurosurgery
Fetal bovine serum
Subjects
Details
- Language :
- English
- ISSN :
- 01418955 and 15732665
- Database :
- OpenAIRE
- Journal :
- Journal of Inherited Metabolic Disease, Journal of Inherited Metabolic Disease, 2019, ⟨10.1002/jimd.12161⟩, Journal of Inherited Metabolic Disease, Springer Verlag, 2019, ⟨10.1002/jimd.12161⟩, Journal of Inherited Metabolic Disease, Springer Verlag, 2019, 214 (12), pp.201817815. ⟨10.1002/jimd.12161⟩
- Accession number :
- edsair.doi.dedup.....0fc6c15a6e3ead5ae272f2f6a2e0ad76