• 1
    Sprong H, Degroote S, Claessens T, van Drunen J, Oorschot V, Westerink BH, et al. Glycosphingolipids are required for sorting melanosomal proteins in the Golgi complex. J Cell Biol 2001; 155: 369-380.
  • 2
    Tamboli IY, Prager K, Barth E, Heneka M, Sandhoff K, Walter J. Inhibition of glycosphingolipid biosynthesis reduces secretion of the beta-amyloid precursor protein and amyloid beta-peptide. J Biol Chem 2005; 280: 28110-28117.
  • 3
    Yamashita T, Hashiramoto A, Haluzik M, Mizukami H, Beck S, Norton A, et al. Enhanced insulin sensitivity in mice lacking ganglioside GM3. Proc Natl Acad Sci U S A 2003; 100: 3445-3449.
  • 4
    Liu Y, Su Y, Wiznitzer M, Epifano O, Ladisch S. Ganglioside depletion and EGF responses of human GM3 synthase-deficient fibroblasts. Glycobiology 2008; 18: 593-601.
  • 5
    Wang R, Salem M, Yousef IM, Tuchweber B, Lam P, Childs SJ, et al. Targeted inactivation of sister of P-glycoprotein gene (spgp) in mice results in nonprogressive but persistent intrahepatic cholestasis. Proc Natl Acad Sci U S A 2001; 98: 2011-2016.
  • 6
    Gerloff T, Stieger B, Hagenbuch B, Madon J, Landmann L, Roth J, et al. The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver. J Biol Chem 1998; 273: 10046-10050.
  • 7
    Strautnieks SS, Bull LN, Knisely AS, Kocoshis SA, Dahl N, Arnell H, et al. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis. Nat Genet 1998; 20: 233-238.
  • 8
    Small DM. Role of ABC transporters in secretion of cholesterol from liver into bile. Proc Natl Acad Sci U S A 2003; 100: 4-6.
  • 9
    Yu L, Hammer RE, Li-Hawkins J, Von Bergmann K, Lutjohann D, Cohen JC, et al. Disruption of Abcg5 and Abcg8 in mice reveals their crucial role in biliary cholesterol secretion. Proc Natl Acad Sci U S A 2002; 99: 16237-16242.
  • 10
    Oram JF, Lawn RM, Garvin MR, Wade DP. ABCA1 is the cAMP-inducible apolipoprotein receptor that mediates cholesterol secretion from macrophages. J Biol Chem 2000; 275: 34508-34511.
  • 11
    Bodzioch M, Orso E, Klucken J, Langmann T, Bottcher A, Diederich W, et al. The gene encoding ATP-binding cassette transporter 1 is mutated in Tangier disease. Nat Genet 1999; 22: 347-351.
  • 12
    Glaros EN, Kim WS, Quinn CM, Wong J, Gelissen I, Jessup W, et al. Glycosphingolipid accumulation inhibits cholesterol efflux via the ABCA1/apolipoprotein A-I pathway: 1-phenyl-2-decanoylamino-3-morpholino-1-propanol is a novel cholesterol efflux accelerator. J Biol Chem 2005; 280: 24515-24523.
  • 13
    Bijl N, Scheij S, Houten S, Boot RG, Groen AK, Aerts JM. The glucosylceramide synthase inhibitor N-(5-adamantane-1-yl-methoxy-pentyl)-deoxynojirimycin induces sterol regulatory element-binding protein-regulated gene expression and cholesterol synthesis in HepG2 cells. J Pharmacol Exp Ther 2008; 326: 849-855.
  • 14
    Bijl N, van Roomen CP, Triantis V, Sokolovic M, Ottenhoff R, Scheij S, et al. Reduction of glycosphingolipid biosynthesis stimulates biliary lipid secretion in mice. HEPATOLOGY 2009; 49: 637-645.
  • 15
    Jennemann R, Sandhoff R, Langbein L, Kaden S, Rothermel U, Gallala H, et al. Integrity and barrier function of the epidermis critically depend on glucosylceramide synthesis. J Biol Chem 2007; 282: 3083-3094.
  • 16
    Jennemann R, Sandhoff R, Wang S, Kiss E, Gretz N, Zuliani C, et al. Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth. Proc Natl Acad Sci U S A 2005; 102: 12459-12464.
  • 17
    Postic C, Magnuson MA. [Role of glucokinase (GK) in the maintenance of glucose homeostasis. Specific disruption of the gene by the Cre-loxP technique]. J Annu Diabetol Hotel Dieu 1999: 115-124.
  • 18
    Chomczynski P, Sacchi N. Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem 1987; 162: 156-159.
  • 19
    Ghauharali-van der Vlugt K, Langeveld M, Poppema A, Kuiper S, Hollak CE, Aerts JM, et al. Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease. Clin Chim Acta 2008; 389: 109-113.
  • 20
    Groener JE, Poorthuis BJ, Kuiper S, Helmond MT, Hollak CE, Aerts JM. HPLC for simultaneous quantification of total ceramide, glucosylceramide, and ceramide trihexoside concentrations in plasma. Clin Chem 2007; 53: 742-747.
  • 21
    Neville DC, Coquard V, Priestman DA, te Vruchte DJ, Sillence DJ, Dwek RA, et al. Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling. Anal Biochem 2004; 331: 275-282.
  • 22
    Sandhoff R, Geyer R, Jennemann R, Paret C, Kiss E, Yamashita T, et al. Novel class of glycosphingolipids involved in male fertility. J Biol Chem 2005; 280: 27310-27318.
  • 23
    Sandhoff R, Hepbildikler ST, Jennemann R, Geyer R, Gieselmann V, Proia RL, et al. Kidney sulfatides in mouse models of inherited glycosphingolipid disorders: determination by nano-electrospray ionization tandem mass spectrometry. J Biol Chem 2002; 277: 20386-20398.
  • 24
    Out R, Hoekstra M, Habets K, Meurs I, de Waard V, Hildebrand RB, et al. Combined deletion of macrophage ABCA1 and ABCG1 leads to massive lipid accumulation in tissue macrophages and distinct atherosclerosis at relatively low plasma cholesterol levels. Arterioscler Thromb Vasc Biol 2008; 28: 258-264.
  • 25
    Out R, Jessup W, Le Goff W, Hoekstra M, Gelissen IC, Zhao Y, et al. Coexistence of foam cells and hypocholesterolemia in mice lacking the ABC transporters A1 and G1. Circ Res 2008; 102: 113-120.
  • 26
    Pan B, Fromholt SE, Hess EJ, Crawford TO, Griffin JW, Sheikh KA, et al. Myelin-associated glycoprotein and complementary axonal ligands, gangliosides, mediate axon stability in the CNS and PNS: neuropathology and behavioral deficits in single- and double-null mice. Exp Neurol 2005; 195: 208-217.
  • 27
    Ikonen E. Cellular cholesterol trafficking and compartmentalization. Nat Rev Mol Cell Biol 2008; 9: 125-138.
  • 28
    Lo Sasso G, Petruzzelli M, Moschetta A. A translational view on the biliary lipid secretory network. Biochim Biophys Acta 2008; 1781: 79-96.
  • 29
    Aerts JM, Ottenhoff R, Powlson AS, Grefhorst A, van Eijk M, Dubbelhuis PF, et al. Pharmacological inhibition of glucosylceramide synthase enhances insulin sensitivity. Diabetes 2007; 56: 1341-1349.
  • 30
    Zhao H, Przybylska M, Wu IH, Zhang J, Maniatis P, Pacheco J, et al. Inhibiting glycosphingolipid synthesis ameliorates hepatic steatosis in obese mice. HEPATOLOGY 2009; 50: 85-93.
  • 31
    Bijl N, Sokolovic M, Vrins C, Langeveld M, Moerland PD, Ottenhoff R, et al. Modulation of glycosphingolipid metabolism significantly improves hepatic insulin sensitivity and reverses hepatic steatosis in mice. HEPATOLOGY 2009; 50: 1431-1441.