Artikel ; Online: Determination of globotriaosylceramide analogs in the organs of a mouse model of Fabry disease.
The Journal of biological chemistry
2020 Band 295, Heft 17, Seite(n) 5577–5587
Abstract: Fabry disease is a heritable lipid disorder caused by the low activity of α-galactosidase A and characterized by the systemic accumulation of globotriaosylceramide (Gb3). Recent studies have reported a structural heterogeneity of Gb3 in Fabry disease, ... ...
Abstract | Fabry disease is a heritable lipid disorder caused by the low activity of α-galactosidase A and characterized by the systemic accumulation of globotriaosylceramide (Gb3). Recent studies have reported a structural heterogeneity of Gb3 in Fabry disease, including Gb3 isoforms with different fatty acids and Gb3 analogs with modifications on the sphingosine moiety. However, Gb3 assays are often performed only on the selected Gb3 isoforms. To precisely determine the total Gb3 concentration, here we established two methods for determining both Gb3 isoforms and analogs. One was the deacylation method, involving Gb3 treatment with sphingolipid ceramide |
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Mesh-Begriff(e) | Acylation ; Animals ; Chromatography, High Pressure Liquid ; Disease Models, Animal ; Fabry Disease/pathology ; Humans ; Kidney/pathology ; Liver/pathology ; Male ; Mice ; Myocardium/pathology ; Spleen/pathology ; Tandem Mass Spectrometry ; Trihexosylceramides/analysis |
Chemische Substanzen | Trihexosylceramides ; globotriaosylceramide (71965-57-6) |
Sprache | Englisch |
Erscheinungsdatum | 2020-03-16 |
Erscheinungsland | United States |
Dokumenttyp | Journal Article |
ZDB-ID | 2997-x |
ISSN | 1083-351X ; 0021-9258 |
ISSN (online) | 1083-351X |
ISSN | 0021-9258 |
DOI | 10.1074/jbc.RA120.012665 |
Datenquelle | MEDical Literature Analysis and Retrieval System OnLINE |
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