Sphingolipids: The nexus between Gaucher disease and insulin resistance

Lipids in Health and Disease

Volumn 9, Issue , 2010, Pages

  Fuller, Maria ^a,b  

^a WOMEN S AND CHILDREN S HOSPITAL   (Australia)

^b UNIVERSITY OF ADELAIDE   (Australia)

Author keywords

[No Author keywords available]

Indexed keywords

CERAMIDE; GANGLIOSIDE; GLUCOSYLCERAMIDE; GLYCOSPHINGOLIPID; SPHINGOLIPID; SPHINGOMYELIN;

CELL MEMBRANE; GAUCHER DISEASE; HUMAN; INSULIN RESISTANCE; INSULIN SENSITIVITY; LIPID COMPOSITION; LIPID METABOLISM; LIPID RAFT; PROTEIN FUNCTION; PROTEIN INTERACTION; PROTEIN STRUCTURE; PROTEIN TARGETING; REVIEW; SIGNAL TRANSDUCTION; TIGHT JUNCTION; METABOLISM; PHYSIOLOGY;

EUKARYOTA;

GAUCHER DISEASE; GLUCOSYLCERAMIDES; HUMANS; INSULIN RESISTANCE; SPHINGOLIPIDS;

EID: 77957652713     PISSN: None     EISSN: 1476511X     Source Type: Journal    
DOI: 10.1186/1476-511X-9-113     Document Type: Review

References (110)

1. The complex life of simple sphingolipids. AH Futerman YA Hannun, EMBO reports 2004 5 777 782 10.1038/sj.embor.7400208 15289826
2. Sphingolipid functions in Saccharomyces cerevisiae: Comparison to mammals. RC Dickson, Ann Rev Biochem 1998 67 27 48 10.1146/annurev.biochem.67.1. 27 9759481
3. Glycosphingolipid structures. RK Yu M Yanagisawa T Ariga, Comprehensive Glycoscience Elseiver: Oxford, Kamerling JP, 2007 73 122 full-text
4. Combinatorial ganglioside biosynthesis. T Kolter RL Proia K Sandhoff, J Biol Chem 2002 277 25859 25862 10.1074/jbc.R200001200 12011101
5. Sphingolipid metabolism diseases. T Kolter K Sandhoff, Biochim Biophys Acta 2006 1758 2057 2079 10.1016/j.bbamem.2006.05.027 16854371
6. Glycosphingolipidoses: beyond the enzymatic defect. A Raas-Rothschild I Pankova-Kholmyansky Y Kacher AH Futerman, Glycoconj J 2004 21 295 304 10.1023/B:GLYC.0000046272.38480.ef 15514478
7. Gaucher disease. E Beutler GA Grabowski, The Metabolic and Molecular Basis of Inherited Disease McGraw-Hill: New York, Scriver CR, Beaudet AL, Sly WS, 2001 3635 3668
8. Secondary sphingolipid accumulation in a macrophage model of Gaucher disease. LK Hein PJ Meikle JJ Hopwood M Fuller, Mol Genet Metab 2007 92 336 345 10.1016/j.ymgme.2007.08.001 17881272
9. Glucosylceramide is not confined to the lysosome in fibroblasts from patients with Gaucher disease. M Fuller T Rozaklis M Lovejoy K Zarrinakalam JJ Hopwood PJ Meikle, Mol Genet Metab 2008 93 437 443 10.1016/j.ymgme.2007.11.011 18162427
10. Glycosphingolipids and insulin resistance. M Langeveld JMFG Aerts, Prog Lipid Res 2009 48 196 205 10.1016/j.plipres.2009.03.002 19303901
11. The ceramide-centric universe of lipid-mediated cell regulation: stress encounters of the lipid kind. YA Hannun LM Obeid, J Biol Chem 2002 277 25847 25850 10.1074/jbc.R200008200 12011103
12. Simulation and evaluation of sphingolipid metabolism in S. cerevisae. F Alvarez-Vasquez KJ Sims Y Cowart Y Okamoto EO Voit YA Hannun, Nature 2005 433 425 429 10.1038/nature03232 15674294
13. Characterisation of serine palmitoyltransferase activity in Chinese hamster ovary cells. A Merrill, Biochim Biophys Acta 1983 754 284 291 6652105
14. Kinetics of long-chain (sphingoid) base biosynthesis in intact LM cells: effects of varying the extracellular concentrations of serine and fatty acid precursors of this pathway. AH Merrill Jr E Wang RE Mullins, Biochemistry 1988 12 340 345 10.1021/bi00401a051
15. Sphingolipids in inflammation: roles and implications. BJ Pettus CE Chalfant YA Hannun, Curr Mol Med 2004 4 405 418 10.2174/1566524043360573 15354871
16. When do Lasses (longevity assurance genes) become CerS (ceramide synthases)?: insights into the regulation of ceramide synthesis. Y Pewzner-Jung S Ben-Dor AH Futerman, J Biol Chem 2006 281 25001 25005 10.1074/jbc.R600010200 16793762
17. Characterisation of ceramide synthesis. A dihydroceramide desaturase introduces the 4,5-trans-double bond of sphingosine at the level of dihydroceramide. C Michel G van Echten-Deckert J Rother K Sandhoff E Wang AH Merrill Jr, J Biol Chem 1997 272 22432 22437 10.1074/jbc.272.36.22432 9312549
18. Sphingosine kinases, sphingosine 1-phosphate, apotosis and diseases. NC Hait CA Oskeritzian SW Paugh S Milstien S Spiegel, Biochim Biophys Acta 2006 1758 2016 2026 10.1016/j.bbamem.2006.08.007 16996023
19. Ceramide kinase, a novel lipid kinase. Molecular cloning and functional characterisation. M Sugiura K Kono H Liu T Shimizugawa H Minekura S Spiegel T Kohama, J Biol Chem 2002 277 23294 23300 10.1074/jbc.M201535200 11956206
20. The subcellular sites of sphingomyelin synthesis in BHK cells. JM Mira Obradors D Sillence S Howitt D Allan, Biochim Biophys Acta 1997 1359 1 12 10.1016/S0167-4889(97)00088-8 9398080
21. Glucosylceramide synthase and glycosphingolipid synthesis. S Ichikawa Y Hirabayashi, Trends Cell Biol 1998 8 198 202 10.1016/S0962-8924(98)01249-5 9695839
22. Purification, cDNA cloning and expression of UDP-Gal: glucosylceramide beta-1,4-galactosyltransferase from rat brain. T Nomura M Takizawa J Aoki H Arai K Inoue E Wakisaka N Yoshizuka G Imokawa N Dohmae K Takio M Hattori N Matsuo, J Biol Chem 1998 273 13570 13577 10.1074/jbc.273.22.13570 9593693
23. Gangliosides of human myelin: sialosylgalactosylceramide (G7) as a major component. RW Ledeen RK Yu LF Eng, J Neurochem 1973 21 829 839 10.1111/j.1471-4159.1973.tb07527.x 4754859
24. Biosynthesis and degradation of mammalian glycosphingolipids. K Sandhoff T Kolter, Phil Trans R Soc Lond B 2003 358 847 861 10.1098/rstb.2003.1265 (Pubitemid 36639708)
25. Lysosomal storage diseases. EF Neufeld, Ann Rev Biochem 1991 60 257 280 10.1146/annurev.bi.60.070191.001353 1883197
26. Pathways of clathrin-independent endocytosis. S Mayor RE Pagano, Nat Rev Mol Cell Biol 2007 8 603 612 10.1038/nrm2216 17609668
27. Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP. G Wilkening T Linke G Uhlhorn-Dierks K Sandhoff, J Biol Chem 2000 275 35814 35819 10.1074/jbc.M006568200 10942779
28. Over-expression of a functionally active human GM2-activator protein in Escherichia Coli. H Klima A Klein G van Echten G Schwarzmann K Suzuki K Sandhoff, Biochem J 1993 292 Pt 2 571 576 8503891
29. Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP) precursor in cultured human fibroblasts. Mannose 6-phosphate receptor-independent endocytosis of SAP precursor. G Vielhaber R Hurwitz K Sandhoff, J Biol Chem 1996 271 32438 32446 10.1074/jbc.271.50.32438 8943309
30. Degradation of gangliosides by the lysosomal sialidase requires an activator protein. R Fingerhut GT Van der Horst FW Verheijen E Conzelmann, Eur J Biochem 1992 208 623 629 10.1111/j.1432-1033.1992.tb17227.x 1396669
31. Hydrolysis of lactosylceramide by human galactosylceramidase and GM1 - galactosidase in a detergent free system and its stimulation by sphingolipid activator proteins, sap-B and sap-C. A Zschoche W Fürst G Schwarzmann K Sandhoff, Eur J Biochem 1994 222 83 90 10.1111/j.1432-1033.1994.tb18844.x 8200356
32. Stimulation of lysosomal sphingomyelin degradation by sphingolipid activator proteins. K Ferlinz T Linke O Bartelsen M Weiler K Sandhoff, Chem Phys Lipids 1999 102 35 43 10.1016/S0009-3084(99)00073-0 11001559
33. Interfacial regulation of acid ceramidase activity. T Linke G Wilkening F Sadeghlar H Moczall K Bernardo E Schuchman K Sandhoff, J Biol Chem 2001 276 5760 5768 10.1074/jbc.M006846200 11104761
34. Roles and regulation of secretory and lysosomal acid sphingomyelinase. RW Jenkins D Canals YA Hannun, Cell Signal 2009 21 836 846 10.1016/j.cellsig.2009. 01.026 19385042
35. Regulation of lipid metabolism by sphingolipids. TS Worgall, Subcell Biochem 2008 49 371 385 full-text 18751919
36. Bioactive sphingolipids: metabolism and function. N Bartke YA Hannun, J Lipid Res 2009 50 91 96 10.1194/jlr.R800080-JLR200 19017611
37. Principles of bioactive lipid signaling: lessons from sphingolipids. YA Hannun LM Obeid, Nat Rev Mol Cell Biol 2008 9 139 150 10.1038/nrm2329 18216770
38. Programmed cell death induced by ceramide. LM Obeid CM Linardic LA Karolak YA Hannun, Science 1993 259 1769 1771 10.1126/science.8456305 8456305
39. Role of ceramide in cellular senescence. ME Venable JY Lee MJ Smyth A Bielawska LM Obeid, J Biol Chem 1995 270 30701 30708 10.1074/jbc.270.51.30701 8530509
40. Effects of sphingosine and other sphingolipids on protein kinase C. ER Smith AH Merrill LM Obeid YA Hannun, Methods Enzymol 2000 312 61 373
41. Physiological and pathological actions of sphingosine 1-phosphate. T Hia, Semin Cell Dev Biol 2004 15 513 520 10.1016/j.semcdb.2004.05.002 15271296
42. Sphingosine 1-phosphate and ceramide 1-phosphate: expanding roles in cell signalling. CE Chalfant S Spiegel, J Cell Sci 2005 118 4605 4612 10.1242/jcs.02637 16219683
43. Glycosphingolipids and drug resistance. V Gouaze-Anderson MC Cabot, Biochim Biophys Acta 2006 1758 2096 2103 10.1016/j.bbamem.2006.08.012 17010304
44. Ceramide synthase inhibition by fumonisin B1 causes accumulation of 1-deoxysphinganine: a novel category of bioactive 1-deoxysphingoid bases and 1-deoxydihydroceramides biosynthesised by mammalian cell lines and animals. NC Zitomer T Mitchell KA Voss GS Bondy ST Pruett EC Garnier-Amblard LS Liebeskind E Park Wang MC Sullards AH Merrill Jr RT Riley, J Biol Chem 2009 284 4786 4795 10.1074/jbc.M808798200 19095642
45. Direct binding to ceramide activates protein kinase Czeta before the formation of a pro-apoptotic complex with PAR-4 in differentiating stem cells. G Wang J Silva K Krishnamurthy E Tran BG Condie E Bieberich, J Biol Chem 2005 280 26415 26424 10.1074/jbc.M501492200 15901738
46. Glycosphingolipids in cellular interaction, differentiation, oncogenesis. S Hakomori, Ann Rev Biochem 1981 50 733 764 10.1146/annurev.bi.50.070181.003505 7023369
47. Functional rafts in cell membranes. K Simons E Ikonen, Nature 1997 387 569 592 10.1038/42408 9177342
48. Rafts defined: a report on the Keystone symposium on lipid rafts and cell function. LJ Pike, J Lipid Res 2006 47 1597 1598 10.1194/jlr.E600002-JLR200 16645198
49. Lipid composition of microdomains is altered in a cell model of Gaucher disease. LK Hein S Duplock JJ Hopwood M Fuller, J Lipid Res 2008 49 1725 1734 10.1194/jlr.M800092-JLR200 18427156
50. Lipid rafts and signal transduction. K Simons D Toomre, Nat Rev Mol Cell Biol 2000 1 31 41 10.1038/35036052 11413487
51. Lipid rafts: elusive or illusive? S Munro, Cell 2003 115 377 388 10.1016/S0092-8674(03)00882-1 14622593
52. Spatial and temporal control of signaling through lipid rafts. T Golub S Wacha P Caroni, Curr Opin Neurobiol 2004 14 542 550 10.1016/j.conb.2004.08.003 15464886
53. Lipid rafts in health and disease. V Michel M Bakovic, Biol Cell 2007 99 129 140 10.1042/BC20060051 17064251
54. Raft domains contribute to Akt/PKB plasma membrane recruitment and activation. R Lasserre X-J Guo F Conchonaud Y Hamon O Hawchar A-M Bernard SM Soudja P-F Lenne H Rigneault D Olive G Bismuth JA Nunès B Payraste D Marguet H-T He, Nat Chem Biol 2008 4 538 547 10.1038/nchembio.103 18641634
55. Characterisation of a 3-phosphoinositide-dependent protein kinase which phosphorylates and activates protein kinase Balpha. DR Alessi SR James CP Downes AB Holmes PR Gaffney CB Reese P Cohen, Curr Biol 1997 7 261 269 10.1016/S0960-9822(06)00122-9 9094314
56. Phosphorylation and regulation of Akt/PKB by the rector-mTOR complex. DD Sarbassov DA Guertin SM Ali DM Sabatini, Science 2005 307 1098 1101 10.1126/science.1106148 15718470
57. The protein kinase B/Akt signaling pathway in human malignancy. KM Nicholson NG Anderson, Cell Signal 2002 14 381 395 10.1016/S0898-6568(01)00271-6 11882383
58. Spatiotemporal analysis of differential Akt regulation in plasma membrane microdomains. X Gao J Zhang, Mol Biol Cell 2008 19 4366 4373 10.1091/mbc.E08-05-0449 18701703
59. The cell biology of lysosomal storage disorders. AH Futerman G van Meer, Nat Rev Mol Cell Biol 2004 5 554 565 10.1038/nrm1423 15232573
60. Broad screening test for sphingolipid-storage diseases. CS Chen MC Patterson CL Wheatley JF O'Brien RE Pagano, Lancet 1999 354 901 905 10.1016/S0140-6736(98)10034-X 10489949
61. Effect of lysosomal storage on bis(monoacylglycero)phosphate. PJ Meikle S Duplock D Blacklock PD Whitfield G MacIntosh JJ Hopwood M Fuller, Biochem J 2008 411 71 78 10.1042/BJ20071043 18052935
62. Jamming the endosomal system: lipid rafts and lysosomal storage diseases. K Simons J Gruenberg, Trends Cell Biol 2000 10 459 462 10.1016/S0962-8924(00) 01847-X 11050411
63. Depletion of rafts in late endocytic membranes is controlled by NPC-dependent recycling of cholesterol to the plasma membrane. S Lusa TS Blom EL Eskelinen E E. Kuismanen JE Mansson K Simons E Ikonen, J Cell Sc 2001 114 1893 1900
64. Prevalence of lysosomal storage disorders. PJ Meikle JJ Hopwood AE Clague WF Carey, JAMA 1999 281 249 254 10.1001/jama.281.3.249 9918480
65. De l'épithélioma primitive de la rate, hypertrophie idiopathique de la rate sans leucémie. PCE Gaucher, Paris, MD Thesis 1882
66. Replacement therapy for inherited enzyme deficiency-macrophage-targeted glucocerebrosidase for Gaucher's disease. NW Barton RO Brady JM Dambrosia AM Di Bisceglie SH Doppelt SC Hill HJ Mankin GJ Murray RI Parker CE Argoff, et al. N Engl J Med 1991 324 1464 1470 10.1056/NEJM199105233242104 2023606
67. Effectiveness of enzyme replacement therapy in 1028 patients with type 1 Gaucher disease after 2 to 5 years of treatment: a report from the Gaucher Registry. NJ Weinreb J Charrow HC Andersson P Kaplan EH Kolodny P Mistry G Pastores BE Rosenbloom CR Scott RS Wappner A Zimran, Am J Med 2002 113 112 119 10.1016/S0002-9343(02)01150-6 12133749 (Pubitemid 34804747)
68. Cell biology and biochemistry of acid -glucosidase: the Gaucher disease enzyme. GA Grabowski A Kazimierczuk B Liou, Gaucher disease Boca Raton: CRC Press, Futerman AH, Zimran A, 2006 49 66
69. Analysis of variant acid -glucosidases: effects of Gaucher disease mutations. B Liou A Kazimierczuk M Zhang CR Scott RS Hedge GA Grabowski, J Biol Chem 2006 281 4242 4253 10.1074/jbc.M511110200 16293621
70. Gaucher disease: pathological mechanisms and modern management. M Jmoudiak AH Futerman, Br J Haematol 2005 129 178 188 10.1111/j.1365-2141.2004. 05351.x 15813845
71. Lysosomal storage disease 1. GA Grabowski, Lancet 2008 372 1263 1271 10.1016/S0140-6736(08)61522-6 19094956
72. Gaucher disease: unmet treatment needs. A Mehta, Acta Paediatr Suppl 2008 97 83 87 10.1111/j.1651-2227.2008.00653.x 18339195
73. Glycolipid analysis of different tissues and cerebrospinal fluid in type II Gaucher disease. R Gornati B Berra G Montorfano C Martini G Ciana P Ferrari M Romano B Bembi, J Inherit Metab Dis 2002 25 47 55 10.1023/A:1015137917508 11999980
74. Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP: phosphocholine cytidylyltransferase by glucosylceramide. J Bodennec D Pelled C Riebeling S Trajkovic AH Futerman, FASEB J 2002 16 1814 1816 12223447
75. Cytokines in Gaucher's disease. V Barak M Acker B Nisman I Kalickman A Abrahamov A Zimran S Yatziv, Eur Cytok Net 1999 10 205 210
76. Musculoskeletal complications associated with lysosomal storage disorders: Gaucher disease and Hurler-Scheie syndrome (mucopolysaccharidosis type 1). GM Pastores PA Meere, Curr Opin Rheumatol 2004 17 70 78 10.1097/01.bor.0000147283.40529.13
77. Sphingosine 1-phosphate mobilises osteoclast precursors and regulates bone homeostasis. M Ishii JG Egan F Klauschen M Meier-Schellersheim Y Saeki J Vacher RL Proia RN Germain, Nature 2009 458 524 529 10.1038/nature07713 19204730
78. Characterisation of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome. PM Campeau M Rafei M Boivin Y Sun GA Grabowski J Galipeau, Blood 2009 114 3181 3190 10.1182/blood-2009-02-205708 19587377
79. The pathogenesis of glycosphingolipid storage disorders. L Ginzburg Y Kacher AH Futerman, Semin Cell Dev Biol 2004 15 417 431 10.1016/j.semcdb.2004. 03.003 15207832
80. Glucocerebroside: an evolutionary advantage for patients with Gaucher disease and a new immunomodulatory agent. Y Ilan D Elstein A Zimran, Immunol Cell Biol 2009 87 514 524 10.1038/icb.2009.42 19529001
81. Autophagy regulates lipid metabolism. R Singh S Kaushik Y Wang Y Xiang I Novak M Komatsu K Tanaka AM Cuervo MJ Czaja, Nature 2009 458 1131 1137 10.1038/nature07976 19339967
82. Autophagy and lipids: tightening the knot. JA Rodriguez-Navarro AM Cuervo, Semin Immunopathol 2010 20730586
83. Molecular mechanisms of insulin resistance. S Schinner WA Scherbaum RS Bornstein A Barthel, Diabetic Med 2005 22 674 682 10.1111/j.1464-5491.2005. 01566.x 15910615
84. Insulin signaling to mTOR mediated by the Akt/PKB substrate PRAS40. E Vander Haar S Lee S Bandhakavi TJ Griffin D-H Kim, Nat Cell Biol 2007 9 316 323 10.1038/ncb1547 17277771
85. Spatial compartmentalisation of signal transduction in insulin action. CA Baumann AR Saltiel, Bioessays 2001 23 215 222 10.1002/1521-1878(200103)23: 3<215::AID-BIES1031>3.0.CO;2-S 11223878
86. Dynamic association of human insulin receptor with lipid rafts in cells lacking caveolae. S Vainio S Heino J Mansson P Fredman K Kuismanen O Vaarala E Ikonen, EMBO Rep 2002 3 95 100 10.1093/embo-reports/kvf010 11751579
87. Regulation of insulin action by ceramide: dual mechanisms linking ceramide accumulation to the inhibition of Akt/protein kinase B. S Stratford KL Hoehn F Liu SA Summers, J Biol Chem 2004 279 36608 36615 10.1074/jbc.M406499200 15220355
88. Ceramide content is increased in skeletal muscle from obese insulin-resistant humans. JM Adams T Pratipanawatr R Berria E Wang RA DeFronzo MC Sullards LJ Mandarino, Diabetes 2004 53 25 31 10.2337/diabetes.53.1.25 14693694
89. Human skeletal muscle ceramide content is not a major factor in muscle insulin sensitivity. M Skovbro M Baranowski C Skov-Jensen A Flint F Dela J Gorski JW Helge, Diabetologia 2008 51 1253 1260 10.1007/s00125-008-1014-z 18458871
90. Inhibition of ceramide synthesis ameliorates glucocorticoid-, saturated-fat-, and obesity-induced insulin resistance. WL Holland JT Broznick LP Wang ED Hawkins KM Sargent Y Liu K Narra KL Hoehn TA Knotts A Siesky DH Nelson SK Karathanasis GK Fontenot MJ Birnbaum SA Summers, Cell Metab 2007 5 167 179 10.1016/j.cmet.2007.01.002 17339025
91. Ceramide: a contributor to insulin resistance or an innocent bystander? G Boden, Diabetologia 2008 51 1095 1096 10.1007/s00125-008-1015-y 18458870
92. Enhanced insulin sensitivity in mice lacking ganglioside GM3. T Yamashita A Hashiramoto M Haluzik H Mizukami S Beck A Norton M Kono S Tsuji JL Daniotti N Werth R Sandhoff K Sandhoff RL Proia, PNAS 2003 100 3445 3449 10.1073/pnas.0635898100 12629211
93. Ganglioside GM3 participates in the pathological conditions of insulin resistance. S Tagami JJ Inokuchi K Kabayama H Yoshimura F Kitamura S Uemura C Ogawa A Ishii M Saito Y Ohtsuka S Sakaue Y Igarashi, J Biol Chem 2002 277 3085 3092 10.1074/jbc.M103705200 11707432
94. Dissociation of the insulin receptor and caveolin-1 complex by ganglioside GM3 in the state of insulin resistance. K Kabayama T Sato K Saito N Loberto A Prinetti S Sonnino M Kinjo Y Igarashi J Inokuchi, PNAS 2007 104 13678 13683 10.1073/pnas.0703650104 17699617
95. Altered adipose and plasma sphingolipid metabolism in obesity: a potential mechanism for cardiovascular and metabolic risk. F Samad KD Hester G Yang YA Hannun J Bielawski, Diabetes 2006 55 2579 2587 10.2337/db06-0330 16936207
96. Type 1 Gaucher disease, a glycosphingolipid storage disorder, is associated with insulin resistance. M Langeveld KJM Ghauharali HP Sauerwein MT Ackermans JEM Groener CEM Hollak JM Aerts MJ Serlie, J Clin Endocrinol Metab 2008 93 845 851 10.1210/jc.2007-1702 18089699
97. Overweight, insulin resistance and type II diabetes in type I Gaucher disease patients in relation to enzyme replacement therapy. M Langeveld M de Fost JMFG Aerts HP Sauerwein CEM Hollak, Blood Cells Mol Dis 2008 40 428 432 10.1016/j.bcmd.2007.09.002 17950007
98. A cross-sectional, mono-centric pilot study of insulin resistance in enzyme replacement therapy patients with Gaucher type 1 without overweight. SK Ucar M Coker M Argin S Akman S Kara DG Simsek S Darcan, Mol Genet Metab 2009 96 50 51 10.1016/j.ymgme.2008.10.001 19006678
99. Lysosomal dysfunction results in altered energy balance. JC Woloszynek T Coleman CF Semenkovich MS Sands, J Biol Chem 2007 282 35765 35771 10.1074/jbc.M705124200 17911106
100. Resting energy expenditure in Gaucher's disease type 1: effect of Gaucher's cell burden on energy requirements. DJ Barton MD Ludman K Benkov GA Grabowski NS LeLeiko, Metabolism 1989 38 1238 1243 10.1016/0026-0495(89)90165-0 2593834
101. Increased basal glucose production in type 1 Gaucher's disease. EP Corssmit CE Hollak E Endert MH van Oers HP Sauerwein JA Romijin, J Clin Endocrinol Metab 1995 80 2653 2657 10.1210/jc.80.9.2653 7673407
102. Plasma lipids are elevated in Gaucher disease: Biochemical markers to evaluate therapeutic intervention. PJ Meikle PD Whitfield T Rozaklis D Blacklock S Duplock D Elstein A Zimran E Mengel P Cannell JJ Hopwood M Fuller, Blood Cells Mol Dis 2008 40 420 427 10.1016/j.bcmd.2007.10.004 18054258
103. Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of Gaucher disease. K Ghauharali-van der Vlugt M Langeveld A Poppema S Kuiper CEM Hollak JM Aerts JEM Groener, Clin Chim Acta 2008 389 109 113 10.1016/j.cca.2007.12.001 18164265
104. Lipid rafts and insulin signalling. PE Bickel, Am J Physiol Endocrinol Metab 2002 282 1 E10 11739076
105. Insulin resistance as a membrane microdomain disorder. J Inokuchi, Yakugaku Zasshi 2007 127 579 586 10.1248/yakushi.127.579 17409686
106. Inflammation and insulin resistance. SE Shoelson J Lee AB Goldfine, J Clin Invest 2006 116 1793 1801 10.1172/JCI29069 16823477
107. Obesity induces a phenotypic switch in adipose tissue macrophage polarization. CN Lumeng JL Bodzin AR Saltiel, J Clin Invest 2007 117 175 184 10.1172/JCI29881 17200717
108. Pharmacological inhibition of glucosylceramide synthase enhances insulin sensitivity. JM Aerts R Ottenhoff AS Powlson AM Grefhorst van Ejik PF Dubbelhuis J Aten F Kuipers MJ Serlie T Wennekes JK Sethi S O'Rahilly HS Overkleeft, Diabetes 2007 56 1341 1349 10.2337/db06-1619 17287460
109. Reducing glyosphingolipid content in adipose tissue of obese mice restores insulin sensitivity, adipogenesis and reduces inflammation. M van Ejik J Aten N Biji R Ottenhoff CPAA van Roomen PF Dubbelhuis I Seeman K Ghauharali-van der Vlugt HS Overkleeft C Arbeeny AK Groen JMFG Aerts, PloS One 2009 4 4723 10.1371/journal.pone.0004723 19305508
110. Comprehensive quantitative analysis of bioactive sphingolipids by high-performance liquid chromatography-tandem mass spectrometry. J Bielawski JS Pierce J Snider B Rembiesa ZM Szulc J Bielawski, Methods Mol Biol 2009 579 443 467 full-text 19763489