MALDI-TOF MS applied to apoC-III glycoforms of patients with congenital disorders affecting O-glycosylation. Comparison with two-dimensional electrophoresis.

Abstract

PURPOSE:

The O-glycan abnormalities accompanying some congenital disorders of glycosylation, namely conserved oligomeric Golgi-congenital disorders of glycosylation (COG-CDGs) and ATP6V0A2-CDGs, are mainly detected using electrophoresis methods applied to circulating apolipoprotein C-III. The objective of this study was to evaluate the reliability of MALDI-TOF MS of apoC-III for the detection and characterization of CDG-associated O-glycan defects.

EXPERIMENTAL DESIGN:

plasmas from CDG-negative, COG-CDG, and ATP6V0A2-CDG patients were analyzed and results were compared to those obtained using 2DE followed by Western blot.

RESULTS:

MALDI-TOF of apoC-III allowed to detect various significant O-glycan abnormalities in CDG-patients with emphasis to COG-CDG. Furthermore, in CDG samples, comparison study between 2DE and MALDI-TOF showed a particular behavior of monosialylated apoC-III in the mass spectrometer that could be related to an abnormal O-glycan structure.

CONCLUSIONS AND CLINICAL RELEVANCE:

MALDI-TOF MS appears as a powerful technique for the analysis of apoC-III glycoforms for potential routine screening of COG- and ATP6V0A2-CDGs.

• Authors: Stéphanie Yen-Nicolaÿ, Céline Boursier, Marlène Rio, Dirk J Lefeber, Antoine Pilon, Nathalie Seta, Arnaud Bruneel
• Journal: Proteomics. Clinical applications (Proteomics Clin Appl) Vol. 9 Issue 7-8 Pg. 787-93 (Aug 2015) ISSN: 1862-8354 [Electronic] Germany
• PMID: 25641685 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

Chemical References

• Apolipoprotein C-III
• Glycoproteins
• Mucins
• Polysaccharides

Topics

• Apolipoprotein C-III (metabolism)
• Blotting, Western
• Congenital Disorders of Glycosylation (metabolism)
• Electrophoresis, Gel, Two-Dimensional (methods)
• Glycoproteins (metabolism)
• Humans
• Mucins (chemistry, metabolism)
• Polysaccharides (chemistry, metabolism)
• Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization (methods)