Fabry disease: renal sphingolipid distribution in the alpha-Gal A knockout mouse model by mass spectrometric and immunohistochemical imaging

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F61989592%3A15310%2F15%3A33156414" target="_blank" >RIV/61989592:15310/15:33156414 - isvavai.cz</a>

Alternative codes found

RIV/61388971:_____/15:00444865 RIV/00216208:11110/15:10319214

Result on the web

<a href="http://link.springer.com/article/10.1007%2Fs00216-014-8402-7" target="_blank" >http://link.springer.com/article/10.1007%2Fs00216-014-8402-7</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1007/s00216-014-8402-7" target="_blank" >10.1007/s00216-014-8402-7</a>

Result language

angličtina

Original language name

Fabry disease: renal sphingolipid distribution in the alpha-Gal A knockout mouse model by mass spectrometric and immunohistochemical imaging

Original language description

Fabry disease is an X-linked lysosomal storage disease due to deficient alpha-galactosidase A (alpha-Gal A) activity and the resultant lysosomal accumulation of globotriaosylceramide (Gb3) and related lipids primarily in blood vessels, kidney, heart, and other organs. The renal distribution of stored glycolipid species in the alpha-Gal A knockout mouse model was compared to that in mice to assess relative distribution and absolute amounts of accumulated sphingolipid isoforms. Twenty isoforms of five sphingolipid groups were visualized by mass spectrometry imaging (MSI), and their distribution was compared with immunohistochemical (IHC) staining of Gb3, the major stored glycosphingolipid in consecutive tissue sections. Quantitative bulk lipid analysis of tissue sections was assessed by electrospray ionization with tandem mass spectrometry (ESI-MS/MS). In contrast to the findings in wild-type mice, all three analytical techniques (MSI, IHC, and ESI-MS/MS) revealed increases in Gb3 isoforms and ceramide dihexosides (composed mostly of galabiosylceramides), respectively. To our knowledge, this is the first report of the distribution of individual molecular species of Gb3 and galabiosylceramides in kidney sections in Fabry disease mouse. In addition, the spatial distribution of ceramides, ceramide monohexosides, and sphingomyelin forms in renal tissue is presented and discussed in the context of their biosynthesis.

Czech name

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Czech description

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Type

J_x - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

CEP classification

CE - Biochemistry

OECD FORD branch

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Project

Result was created during the realization of more than one project. More information in the Projects tab.

Continuities

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Publication year

2015

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Analytical and Bioanalytical Chemistry

ISSN

1618-2642

e-ISSN

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Volume of the periodical

407

Issue of the periodical within the volume

8

Country of publishing house

DE - GERMANY

Number of pages

9

Pages from-to

2283-2291

UT code for WoS article

000351195800026

EID of the result in the Scopus database

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