期刊
INTERNATIONAL JOURNAL OF MASS SPECTROMETRY
卷 260, 期 2-3, 页码 158-165出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.ijms.2006.09.027
关键词
MALDI-TOF; cluster-TOF-SIMS; imaging mass spectrometry; glycosphingolipid; Fabry disease
Fabry disease is an X-linked disorder of glycosphingolipid metabolism, in which a partial or total deficiency of alpha-galactosidase A, a lysosomal enzyme, results in the progressive accumulation of neutral glycosphingolipids (globotriaosylceramide and digalactosylceramide) in most fluids and tissues of the body. Few information is available about the composition and distribution in tissues of the accumulated glycosphingolipids species. Mass spectrometry imaging is an innovative technique, which can provide pieces of information about the distribution of numerous biological compounds, such as lipids, directly on the tissue sections. MALDI-TOF and cluster-TOF-SIMS imaging approaches were used to study the localization of lipids (cholesterol, cholesterol sulfate, vitamin E, glycosphingolipids...) on skin and kidney sections of patients affected by the Fabry disease. Numerous information on pathophysiology were enlightened by both techniques. (C) 2006 Elsevier B.V. All rights reserved.
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