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

L. Kuchar, H. Faltyskova, L. Krasny, R. Dobrovolny, H. Hulkova, J. Ledvinova, M. Volny, M. Strohalm, K. Lemr, L. Kryspinova, B. Asfaw, J. Rybová, RJ. Desnick, V. Havlicek,

. 2015 ; 407 (8) : 2283-2291. [pub] 20141227

Jazyk angličtina Země Německo

Typ dokumentu časopisecké články, práce podpořená grantem

Perzistentní odkaz   https://www.medvik.cz/link/bmc16000420

Grantová podpora
NT14015 MZ0 CEP - Centrální evidence projektů

Digitální knihovna NLK
Plný text - Článek
Zdroj

E-zdroje Online Plný text

NLK ProQuest Central od 2011-01-01 do Před 1 rokem
Medline Complete (EBSCOhost) od 2003-01-01 do Před 1 rokem
Health & Medicine (ProQuest) od 2011-01-01 do Před 1 rokem

Odkazy

PubMed 25542581
DOI 10.1007/s00216-014-8402-7
Knihovny.cz E-zdroje

Fabry disease is an X-linked lysosomal storage disease due to deficient α-galactosidase A (α-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 α-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.

Citace poskytuje Crossref.org

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