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Beta-glucosidase 2 (GBA2) deficiency leads to zonal glucosylceramide accumulation in the liver

Y Yildiz, H Matern, S Matern, S Weber, R Büttner, T Sauerbruch, F Lammert · Zeitschrift für Gastroenterologie · 2008 · Heft 01

Dokument
1168234
CareLit-ID
Jahr
2008
Publikation
PDF
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Metadaten
DOI
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Bibliografische Angaben

Zeitschrift
Zeitschrift für Gastroenterologie
Autor:innen
Y Yildiz, H Matern, S Matern, S Weber, R Büttner, T Sauerbruch, F Lammert
Ausgabe
Heft 01 / 2008
Seiten
–
Erschienen: 2008-01-23 00:00:00
ISSN
0044-2771

Zusammenfassung

Background and aims: The enzyme beta-glucosidase 2 (GBA2) has been purified and characterized from human liver microsomes. GBA2 is highly conserved and ubiquitously present, and was known to hydrolyze bile acid 3-O-glucosides as endogenous compound in the liver. To gain insight into the pathobiological functions of this enzyme and its substrates, we recently generated Gba2 knockout mice. Methods: Vector construction for Gba2 -/- generation, ES cell transformation, screening and blastocyst injections were carried out according to standard protocols. GBA2 protein expression was determined by immunoblot and immunof…

Schlagworte

GBA2-deficient mice apoptosis zonal glucosylceramide accumulation Beta-Glucosidase Chromatography Blastocyst Zeitschrift für Gastroenterologie