MALDI-TOF MS applied to apoC-III glycoforms of patients with congenital disorders affecting O-glycosylation. Comparison with two-dimensional electrophoresis.
Proteomics Clin Appl
; 9(7-8): 787-93, 2015 Aug.
Article
em En
| MEDLINE
| ID: mdl-25641685
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. EXPERIMENTALDESIGN:
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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Glicoproteínas
/
Eletroforese em Gel Bidimensional
/
Espectrometria de Massas por Ionização e Dessorção a Laser Assistida por Matriz
/
Defeitos Congênitos da Glicosilação
/
Apolipoproteína C-III
Limite:
Humans
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article