期刊
CARBOHYDRATE RESEARCH
卷 398, 期 -, 页码 90-100出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.carres.2014.08.014
关键词
Lysosomal storage diseases; Schindler disease type I; Chip-based nanoelectrospray; Quadrupole time-of-flight mass spectrometry; O-Glycosylated amino acids and peptides
资金
- Romanian National Authority for Scientific Research, UEFISCDI [PN-II-ID-PCE-2011-3-0047, PN-II-RU-TE-2011-2-0008, PN-II-PCCA-2011-142]
- European Commission [A-2010-269256]
- European Social Fund
- [POSDRU/159/1.5/S/137750]
In this study an integrative mass spectrometry (MS) approach based on fully automated chip-nanoelectrospray quadrupole time-of-flight was optimized and applied for the discovery and structural characterization of O-glycopeptides in a fraction from the urine of a patient diagnosed with Schindler disease type I. A mixture of O-glycopeptides extracted and purified from an age matched healthy subject served as the control. 49 glycoforms were discovered in the investigated urine fraction from Schindler disease versus only 14 in control urine. Structures with relevant biological significance, previously not described, such as O-fucosylated tetrasaccharides and chains up to pentadecamers O-linked to serine, threonine, or threonine-proline were identified in the pathological urine and characterized by tandem MS (MS/MS). A number of 29 species discovered here, most of which with long chain glycans, were not previously reported as associated to this condition. All glycopeptides were detected in only 1 min analysis time, with a sample consumption situated in the femtomole range. (C) 2014 Elsevier Ltd. All rights reserved.
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