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Numrering	Referens	Omslagsbild	Hitta
1.	Goetghebuer, T, et al. (författare) Time to Switch to Second-line Antiretroviral Therapy in Children With Human Immunodeficiency Virus in Europe and Thailand 2018 Ingår i: Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. - : Oxford University Press (OUP). - 1537-6591. ; 66:4, s. 594-603 Tidskriftsartikel (refereegranskat)
2.	Crichton, S, et al. (författare) Height and timing of growth spurt during puberty in young people living with vertically acquired HIV in Europe and Thailand 2019 Ingår i: AIDS (London, England). - 1473-5571. ; 33:12, s. 1897-1910 Tidskriftsartikel (refereegranskat)
3.	Marques, André R A, et al. (författare) Glucosylated cholesterol in mammalian cells and tissues: formation and degradation by multiple cellular β-glucosidases. 2016 Ingår i: Journal of Lipid Research. - 1539-7262. ; 57, s. 451-463 Tidskriftsartikel (refereegranskat)abstract The membrane lipid glucosylceramide (GlcCer) is continuously formed and degraded. Cells express two GlcCer-degrading β-glucosidases, GBA and GBA2, located in and outside the lysosome, respectively. Here we demonstrate that through transglucosylation both GBA and GBA2 are able to catalyze in vitro the transfer of glucosyl-moieties from GlcCer to cholesterol, and vice versa. Furthermore, the natural occurrence of 1-O-cholesteryl-β-D-glucopyranoside (GlcChol) in mouse tissues and human plasma is demonstrated using LC-MS/MS and 13C6-labelled GlcChol as internal standard. In cells the inhibition of GBA increases GlcChol, whereas inhibition of GBA2 decreases glucosylated sterol. Similarly, in GBA2-deficient mice GlcChol is reduced. Depletion of GlcCer by inhibition of GlcCer synthase decreases GlcChol in cells and likewise in plasma of inhibitor-treated Gaucher disease patients. In tissues of mice with Niemann-Pick type C, a condition characterized by intralysosomal accumulation of cholesterol, marked elevations in GlcChol occur as well. When lysosomal accumulation of cholesterol is induced in cultured cells, GlcChol is formed via lysosomal GBA. This illustrates that reversible transglucosylation reactions are highly dependent on local availability of suitable acceptors. In conclusion, mammalian tissues contain GlcChol formed by transglucosylation through β-glucosidases using GlcCer as donor. Our findings reveal a novel metabolic function for GlcCer.

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Typ av publikation: tidskriftsartikel (3)

Typ av innehåll: refereegranskat (3)

Författare/redaktör: Giacomet, V (2); Maccabruni, A (2); Mur, A (2); Marczynska, M (2); Wyler, CA (2); Rudin, C (2); visa fler...; Duppenthaler, A (2); Moreno, D. (2); Schmid, P. (2); Perez, C. (2); Weber, R. (2); Alvarez, C. (2); Pantaleo, G (2); Freeman, A (2); Evans, J. (2); Boucher, CAB (2); Romano, A (2); Eisenhut, M (2); Gibb, DM (2); Harper, L (2); Butler, K (2); Mazza, A (2); Cellini, M (2); Lyall, H (2); Sharland, M (2); Sloper, K (2); Reiss, P (2); Hoffmann, M. (2); Mendez, M (2); van Rossum, AMC (2); Garzon, M (2); Saavedra, J (2); Battegay, M (2); Bernasconi, E (2); Hosli, I (2); Vernazza, P (2); Hainaut, M (2); Fortuny, C (2); Aebi-Popp, K (2); de Tejada, BM (2); Fehr, J (2); Grawe, C (2); Perez, A. (2); Martínez, J. (2); Jones, L (2); Bustillo, M (2); Berkhout, B (2); Trkola, A (2); Alvarez, S (2); Hancock, J (2); visa färre...

Lärosäte: Karolinska Institutet (2); Lunds universitet (1)

Språk: Engelska (3)

Forskningsämne (UKÄ/SCB): Medicin och hälsovetenskap (2)

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