| Year |
Citation |
Score |
| 2020 |
Encarnação M, Coutinho MF, Cho SM, Cardoso MT, Ribeiro I, Chaves P, Santos JI, Quelhas D, Lacerda L, Leão Teles E, Futerman AH, Vilarinho L, Alves S. NPC1 silent variant induces skipping of exon 11 (p.V562V) and unfolded protein response was found in a specific Niemann-Pick type C patient. Molecular Genetics & Genomic Medicine. e1451. PMID 32931663 DOI: 10.1002/Mgg3.1451 |
0.305 |
|
| 2020 |
Vardi A, Ben-Dor S, Cho SM, Kalinke U, Spanier J, Futerman AH. Mice defective in interferon signaling help distinguish between primary and secondary pathological pathways in a mouse model of neuronal forms of Gaucher disease. Journal of Neuroinflammation. 17: 265. PMID 32892753 DOI: 10.1186/S12974-020-01934-X |
0.387 |
|
| 2020 |
Blumenreich S, Yaacobi C, Vardi A, Barav OB, Vitner EB, Park H, Wang B, Cheng SH, Sardi SP, Futerman AH. Substrate reduction therapy using Genz-667161 reduces levels of pathogenic components in a mouse model of neuronopathic forms of Gaucher disease. Journal of Neurochemistry. PMID 32743826 DOI: 10.1111/Jnc.15136 |
0.412 |
|
| 2020 |
Zelnik ID, Volpert G, Viiri LE, Kauhanen D, Arazi T, Aalto-Setälä K, Laaksonen R, Futerman AH. Different rates of flux through the biosynthetic pathway for long chain versus very-long chain sphingolipids. Journal of Lipid Research. PMID 32651186 DOI: 10.1194/Jlr.Ra120000984 |
0.32 |
|
| 2020 |
Ventura AE, Varela ARP, Dingjan T, Santos TCB, Fedorov A, Futerman AH, Prieto M, Silva LC. Lipid domain formation and membrane shaping by C24-ceramide. Biochimica Et Biophysica Acta. Biomembranes. 183400. PMID 32565121 DOI: 10.1016/J.Bbamem.2020.183400 |
0.32 |
|
| 2019 |
Zelnik ID, Rozman B, Rosenfeld-Gur E, Ben-Dor S, Futerman AH. A Stroll Down the CerS Lane. Advances in Experimental Medicine and Biology. 1159: 49-63. PMID 31502199 DOI: 10.1007/978-3-030-21162-2_4 |
0.35 |
|
| 2019 |
Zelnik ID, Ventura AE, Kim JL, Silva LC, Futerman AH. The role of ceramide in regulating endoplasmic reticulum function. Biochimica Et Biophysica Acta. Molecular and Cell Biology of Lipids. PMID 31233888 DOI: 10.1016/J.Bbalip.2019.06.015 |
0.323 |
|
| 2019 |
Megyeri M, Prasad R, Volpert G, Sliwa-Gonzalez A, Galih A, Aguilera-Romero A, Riezman H, Barral Y, Futerman AH, Schuldiner M. Yeast ceramide synthases, Lag1 and Lac1, have distinct substrate specificity. Journal of Cell Science. PMID 31164445 DOI: 10.1242/Jcs.228411 |
0.323 |
|
| 2019 |
Paget C, Deng S, Soulard D, Priestman DA, Speca S, von Gerichten J, Speak AO, Saroha A, Pewzner-Jung Y, Futerman AH, Mallevaey T, Faveeuw C, Gu X, Platt FM, Sandhoff R, et al. TLR9-mediated dendritic cell activation uncovers mammalian ganglioside species with specific ceramide backbones that activate invariant natural killer T cells. Plos Biology. 17: e3000169. PMID 30822302 DOI: 10.1371/Journal.Pbio.3000169 |
0.307 |
|
| 2019 |
Kuo CL, Kallemeijn WW, Lelieveld LT, Mirzaian M, Zoutendijk I, Vardi A, Futerman AH, Meijer AH, Spaink HP, Overkleeft HS, Aerts JMFG, Artola M. In vivo inactivation of glycosidases by conduritol B epoxide and cyclophellitol as revealed by activity-based protein profiling. The Febs Journal. PMID 30600575 DOI: 10.1111/Febs.14744 |
0.331 |
|
| 2018 |
Cho SM, Vardi A, Platt N, Futerman AH. Absence of infiltrating peripheral myeloid cells in the brains of mouse models of lysosomal storage disorders. Journal of Neurochemistry. PMID 29900534 DOI: 10.1111/Jnc.14483 |
0.412 |
|
| 2018 |
Weinstock L, Furness AM, Herron S, Smith SS, Sankar S, DeRosa SG, Gao D, Mepyans ME, Scotto Rosato A, Medina DL, Vardi A, Ferreira NS, Cho SM, Futerman AH, Slaugenhaupt SA, et al. Fingolimod Phosphate Inhibits Astrocyte Inflammatory Activity in Mucolipidosis IV. Human Molecular Genetics. PMID 29771310 DOI: 10.1093/Hmg/Ddy182 |
0.349 |
|
| 2018 |
Tidhar R, Zelnik ID, Volpert G, Ben-Dor S, Kelly S, Merrill AH, Futerman AH. Eleven residues determine the acyl chain specificity of ceramide synthases. The Journal of Biological Chemistry. PMID 29632068 DOI: 10.1074/Jbc.Ra118.001936 |
0.313 |
|
| 2018 |
Fuller M, Futerman AH. The brain lipidome in neurodegenerative lysosomal storage disorders. Biochemical and Biophysical Research Communications. PMID 29524416 DOI: 10.1016/J.Bbrc.2018.03.042 |
0.358 |
|
| 2018 |
Eisenberg-Bord M, Mari M, Weill U, Rosenfeld-Gur E, Moldavski O, Castro IG, Soni KG, Harpaz N, Levine TP, Futerman AH, Reggiori F, Bankaitis VA, Schuldiner M, Bohnert M. Identification of seipin-linked factors that act as determinants of a lipid droplet subpopulation. The Journal of Cell Biology. 217: 269-282. PMID 29187527 DOI: 10.1083/Jcb.201704122 |
0.306 |
|
| 2017 |
Saroha A, Pewzner-Jung Y, Ferreira NS, Sharma P, Jouan Y, Kelly SL, Feldmesser E, Merrill AH, Trottein F, Paget C, Lang KS, Futerman AH. Critical Role for Very-Long Chain Sphingolipids in Invariant Natural Killer T Cell Development and Homeostasis. Frontiers in Immunology. 8: 1386. PMID 29163475 DOI: 10.3389/Fimmu.2017.01386 |
0.309 |
|
| 2017 |
Kim YR, Volpert G, Shin KO, Kim SY, Shin SH, Lee Y, Sung SH, Lee YM, Ahn JH, Pewzner-Jung Y, Park WJ, Futerman AH, Park JW. Ablation of ceramide synthase 2 exacerbates dextran sodium sulphate-induced colitis in mice due to increased intestinal permeability. Journal of Cellular and Molecular Medicine. PMID 28699686 DOI: 10.1111/Jcmm.13267 |
0.325 |
|
| 2017 |
Dasari SK, Bialik S, Levin-Zaidman S, Levin-Salomon V, Merrill AH, Futerman AH, Kimchi A. Signalome-wide RNAi screen identifies GBA1 as a positive mediator of autophagic cell death. Cell Death and Differentiation. PMID 28574511 DOI: 10.1038/Cdd.2017.80 |
0.334 |
|
| 2017 |
Cingolani F, Simbari F, Abad JL, Casasampere M, Fabrias G, Futerman AH, Casas J. Jaspine B induces non apoptotic cell death in gastric cancer cells independently of its inhibition of ceramide synthase. Journal of Lipid Research. PMID 28572516 DOI: 10.1194/Jlr.M072611 |
0.313 |
|
| 2017 |
Ferreira NS, Engelsby H, Neess D, Kelly SL, Volpert G, Merrill AH, Futerman AH, Faergeman NJ. Regulation of very-long acyl chain ceramide synthesis by Acyl-CoA binding protein. The Journal of Biological Chemistry. PMID 28320857 DOI: 10.1074/Jbc.M117.785345 |
0.38 |
|
| 2017 |
Schonauer S, Körschen HG, Penno A, Rennhack A, Breiden B, Sandhoff K, Gutbrod K, Dörmann P, Raju DN, Haberkant P, Gerl MJ, Brügger B, Zigdon H, Vardi A, Futerman AH, et al. Identification of a feedback loop involving beta-glucosidase 2 and its product sphingosine sheds light on the molecular mechanisms in Gaucher disease. The Journal of Biological Chemistry. PMID 28258214 DOI: 10.1074/Jbc.M116.762831 |
0.38 |
|
| 2017 |
Zigdon H, Meshcheriakova A, Farfel-Becker T, Volpert G, Sabanay H, Futerman AH. Altered lysosome distribution is an early neuropathological event in neurological forms of Gaucher disease. Febs Letters. PMID 28186340 DOI: 10.1002/1873-3468.12591 |
0.382 |
|
| 2017 |
Lebesgue N, Megyeri M, Cristobal A, Scholten A, Chuartzman SG, Voichek Y, Scheltema RA, Mohammed S, Futerman AH, Schuldiner M, Heck AJ, Lemeer S. Combining Deep Sequencing, Proteomics, Phosphoproteomics, and Functional Screens To Discover Novel Regulators of Sphingolipid Homeostasis. Journal of Proteome Research. 16: 571-582. PMID 28152593 DOI: 10.1021/Acs.Jproteome.6B00691 |
0.342 |
|
| 2016 |
Futerman AH, Platt FM. The metabolism of glucocerebrosides - From 1965 to the present. Molecular Genetics and Metabolism. PMID 27955980 DOI: 10.1016/J.Ymgme.2016.11.390 |
0.367 |
|
| 2016 |
Varela AR, Ventura AE, Carreira AC, Fedorov A, Futerman AH, Prieto M, Silva LC. Pathological levels of glucosylceramide change the biophysical properties of artificial and cell membranes. Physical Chemistry Chemical Physics : Pccp. PMID 27905603 DOI: 10.1039/C6Cp07227E |
0.326 |
|
| 2016 |
Hao L, Ben-David O, Babb SM, Futerman AH, Cohen BM, Buttner EA. Clozapine Modulates Glucosylceramide, Clears Aggregated Proteins and Enhances ATG8/LC3 in C. elegans. Neuropsychopharmacology : Official Publication of the American College of Neuropsychopharmacology. PMID 27711049 DOI: 10.1038/Npp.2016.230 |
0.333 |
|
| 2016 |
Megyeri M, Riezman H, Schuldiner M, Futerman AH. Making sense of the yeast sphingolipid pathway. Journal of Molecular Biology. PMID 27664439 DOI: 10.1016/J.Jmb.2016.09.010 |
0.318 |
|
| 2016 |
Klein AD, Ferreira NS, Ben-Dor S, Duan J, Hardy J, Cox TM, Merrill AH, Futerman AH. Identification of Modifier Genes in a Mouse Model of Gaucher Disease. Cell Reports. PMID 27568557 DOI: 10.1016/J.Celrep.2016.07.085 |
0.339 |
|
| 2016 |
Vardi A, Zigdon H, Meshcheriakova A, Klein AD, Yaacobi C, Eilam R, Kenwood BM, Rahim AA, Massaro G, Merrill AH, Vitner EB, Futerman AH. Delineating pathological pathways in a chemically-induced mouse model of Gaucher disease. The Journal of Pathology. PMID 27234572 DOI: 10.1002/Path.4751 |
0.357 |
|
| 2016 |
Vitner EB, Farfel-Becker T, Ferreira NS, Leshkowitz D, Sharma P, Lang KS, Futerman AH. Induction of the type I interferon response in neurological forms of Gaucher disease. Journal of Neuroinflammation. 13: 104. PMID 27175482 DOI: 10.1186/S12974-016-0570-2 |
0.36 |
|
| 2016 |
Cingolani F, Futerman AH, Casas J. Ceramide synthases in biomedical research. Chemistry and Physics of Lipids. 197: 25-32. PMID 26248326 DOI: 10.1016/J.Chemphyslip.2015.07.026 |
0.379 |
|
| 2015 |
Choi S, Kim JA, Kim TH, Li HY, Shin KO, Lee YM, Oh S, Pewzner-Jung Y, Futerman AH, Suh SH. Altering sphingolipid composition with aging induces contractile dysfunction of gastric smooth muscle via KC a 1.1 upregulation. Aging Cell. PMID 26288989 DOI: 10.1111/Acel.12388 |
0.301 |
|
| 2015 |
Ali M, Saroha A, Pewzner-Jung Y, Futerman AH. LPS-mediated septic shock is augmented in ceramide synthase 2 null mice due to elevated activity of TNFα-converting enzyme. Febs Letters. 589: 2213-7. PMID 26183206 DOI: 10.1016/J.Febslet.2015.06.045 |
0.308 |
|
| 2015 |
Park WJ, Brenner O, Kogot-Levin A, Saada A, Merrill AH, Pewzner-Jung Y, Futerman AH. Development of pheochromocytoma in ceramide synthase 2 null mice. Endocrine-Related Cancer. 22: 623-32. PMID 26113602 DOI: 10.1530/Erc-15-0058 |
0.329 |
|
| 2015 |
Zigdon H, Savidor A, Levin Y, Meshcheriakova A, Schiffmann R, Futerman AH. Identification of a biomarker in cerebrospinal fluid for neuronopathic forms of Gaucher disease. Plos One. 10: e0120194. PMID 25775479 DOI: 10.1371/Journal.Pone.0120194 |
0.35 |
|
| 2015 |
Vitner EB, Futerman AH, Platt N. Innate immune responses in the brain of sphingolipid lysosomal storage diseases. Biological Chemistry. 396: 659-67. PMID 25720063 DOI: 10.1515/Hsz-2014-0301 |
0.394 |
|
| 2015 |
Vitner EB, Vardi A, Cox TM, Futerman AH. Emerging therapeutic targets for Gaucher disease. Expert Opinion On Therapeutic Targets. 19: 321-34. PMID 25416676 DOI: 10.1517/14728222.2014.981530 |
0.371 |
|
| 2015 |
Tidhar R, Sims K, Rosenfeld-Gur E, Shaw W, Futerman AH. A rapid ceramide synthase activity using NBD-sphinganine and solid phase extraction. Journal of Lipid Research. 56: 193-9. PMID 25368106 DOI: 10.1194/Jlr.D052001 |
0.317 |
|
| 2014 |
Mosbech MB, Olsen AS, Neess D, Ben-David O, Klitten LL, Larsen J, Sabers A, Vissing J, Nielsen JE, Hasholt L, Klein AD, Tsoory MM, Hjalgrim H, Tommerup N, Futerman AH, et al. Reduced ceramide synthase 2 activity causes progressive myoclonic epilepsy. Annals of Clinical and Translational Neurology. 1: 88-98. PMID 25356388 DOI: 10.1002/Acn3.28 |
0.311 |
|
| 2014 |
Raichur S, Wang ST, Chan PW, Li Y, Ching J, Chaurasia B, Chaurasia B, Dogra S, Öhman MK, Takeda K, Sugii S, Pewzner-Jung Y, Futerman AH, Summers SA. CerS2 haploinsufficiency inhibits β-oxidation and confers susceptibility to diet-induced steatohepatitis and insulin resistance. Cell Metabolism. 20: 687-95. PMID 25295789 DOI: 10.1016/J.Cmet.2014.09.015 |
0.322 |
|
| 2014 |
Varela AR, Gonçalves da Silva AM, Fedorov A, Futerman AH, Prieto M, Silva LC. Influence of intracellular membrane pH on sphingolipid organization and membrane biophysical properties. Langmuir : the Acs Journal of Surfaces and Colloids. 30: 4094-104. PMID 24654655 DOI: 10.1021/La5003397 |
0.306 |
|
| 2014 |
Zigdon H, Meshcheriakova A, Futerman AH. From sheep to mice to cells: tools for the study of the sphingolipidoses. Biochimica Et Biophysica Acta. 1841: 1189-99. PMID 24607565 DOI: 10.1016/J.Bbalip.2014.02.008 |
0.396 |
|
| 2014 |
Vitner EB, Salomon R, Farfel-Becker T, Meshcheriakova A, Ali M, Klein AD, Platt FM, Cox TM, Futerman AH. RIPK3 as a potential therapeutic target for Gaucher's disease Nature Medicine. 20: 204-208. PMID 24441827 DOI: 10.1038/Nm.3449 |
0.393 |
|
| 2014 |
Pinto SN, Laviad EL, Stiban J, Kelly SL, Merrill AH, Prieto M, Futerman AH, Silva LC. Changes in membrane biophysical properties induced by sphingomyelinase depend on the sphingolipid N-acyl chain. Journal of Lipid Research. 55: 53-61. PMID 24163422 DOI: 10.1194/Jlr.M042002 |
0.32 |
|
| 2014 |
Farfel-Becker T, Vitner EB, Kelly SL, Bame JR, Duan J, Shinder V, Merrill AH, Dobrenis K, Futerman AH. Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration. Human Molecular Genetics. 23: 843-54. PMID 24064337 DOI: 10.1093/Hmg/Ddt468 |
0.365 |
|
| 2014 |
Park JW, Park WJ, Futerman AH. Ceramide synthases as potential targets for therapeutic intervention in human diseases. Biochimica Et Biophysica Acta. 1841: 671-81. PMID 24021978 DOI: 10.1016/J.Bbalip.2013.08.019 |
0.358 |
|
| 2013 |
Cohen Y, Megyeri M, Chen OC, Condomitti G, Riezman I, Loizides-Mangold U, Abdul-Sada A, Rimon N, Riezman H, Platt FM, Futerman AH, Schuldiner M. The yeast p5 type ATPase, spf1, regulates manganese transport into the endoplasmic reticulum. Plos One. 8: e85519. PMID 24392018 DOI: 10.1371/Journal.Pone.0085519 |
0.301 |
|
| 2013 |
Halasiddappa LM, Koefeler H, Futerman AH, Hermetter A. Oxidized phospholipids induce ceramide accumulation in RAW 264.7 macrophages: role of ceramide synthases. Plos One. 8: e70002. PMID 23936132 DOI: 10.1371/Journal.Pone.0070002 |
0.336 |
|
| 2013 |
Tidhar R, Futerman AH. The complexity of sphingolipid biosynthesis in the endoplasmic reticulum. Biochimica Et Biophysica Acta. 1833: 2511-8. PMID 23611790 DOI: 10.1016/J.Bbamcr.2013.04.010 |
0.358 |
|
| 2013 |
Vitner EB, Futerman AH. Neuronal forms of Gaucher disease. Handbook of Experimental Pharmacology. 405-19. PMID 23563668 DOI: 10.1007/978-3-7091-1511-4_20 |
0.421 |
|
| 2013 |
Eckl KM, Tidhar R, Thiele H, Oji V, Hausser I, Brodesser S, Preil ML, Onal-Akan A, Stock F, Müller D, Becker K, Casper R, Nürnberg G, Altmüller J, Nürnberg P, ... ... Futerman AH, et al. Impaired epidermal ceramide synthesis causes autosomal recessive congenital ichthyosis and reveals the importance of ceramide acyl chain length. The Journal of Investigative Dermatology. 133: 2202-11. PMID 23549421 DOI: 10.1038/Jid.2013.153 |
0.328 |
|
| 2013 |
Zigdon H, Kogot-Levin A, Park JW, Goldschmidt R, Kelly S, Merrill AH, Scherz A, Pewzner-Jung Y, Saada A, Futerman AH. Ablation of ceramide synthase 2 causes chronic oxidative stress due to disruption of the mitochondrial respiratory chain. The Journal of Biological Chemistry. 288: 4947-56. PMID 23283968 DOI: 10.1074/Jbc.M112.402719 |
0.314 |
|
| 2013 |
Varela AR, Gonçalves da Silva AM, Fedorov A, Futerman AH, Prieto M, Silva LC. Effect of glucosylceramide on the biophysical properties of fluid membranes. Biochimica Et Biophysica Acta. 1828: 1122-30. PMID 23196345 DOI: 10.1016/J.Bbamem.2012.11.018 |
0.326 |
|
| 2012 |
Hullin-Matsuda F, Tomishige N, Sakai S, Ishitsuka R, Ishii K, Makino A, Greimel P, Abe M, Laviad EL, Lagarde M, Vidal H, Saito T, Osada H, Hanada K, Futerman AH, et al. Limonoid compounds inhibit sphingomyelin biosynthesis by preventing CERT protein-dependent extraction of ceramides from the endoplasmic reticulum. The Journal of Biological Chemistry. 287: 24397-411. PMID 22605339 DOI: 10.1074/Jbc.M112.344432 |
0.326 |
|
| 2012 |
Vitner EB, Farfel-Becker T, Eilam R, Biton I, Futerman AH. Contribution of brain inflammation to neuronal cell death in neuronopathic forms of Gaucher's disease. Brain : a Journal of Neurology. 135: 1724-35. PMID 22566609 DOI: 10.1093/Brain/Aws095 |
0.394 |
|
| 2012 |
Laviad EL, Kelly S, Merrill AH, Futerman AH. Modulation of ceramide synthase activity via dimerization. The Journal of Biological Chemistry. 287: 21025-33. PMID 22539345 DOI: 10.1074/Jbc.M112.363580 |
0.315 |
|
| 2012 |
Silva LC, Ben David O, Pewzner-Jung Y, Laviad EL, Stiban J, Bandyopadhyay S, Merrill AH, Prieto M, Futerman AH. Ablation of ceramide synthase 2 strongly affects biophysical properties of membranes. Journal of Lipid Research. 53: 430-6. PMID 22231783 DOI: 10.1194/Jlr.M022715 |
0.308 |
|
| 2011 |
Farfel-Becker T, Vitner EB, Futerman AH. Animal models for Gaucher disease research. Disease Models & Mechanisms. 4: 746-52. PMID 21954067 DOI: 10.1242/Dmm.008185 |
0.357 |
|
| 2011 |
Pinto SN, Silva LC, Futerman AH, Prieto M. Effect of ceramide structure on membrane biophysical properties: the role of acyl chain length and unsaturation. Biochimica Et Biophysica Acta. 1808: 2753-60. PMID 21835161 DOI: 10.1016/J.Bbamem.2011.07.023 |
0.306 |
|
| 2011 |
Ben-David O, Pewzner-Jung Y, Brenner O, Laviad EL, Kogot-Levin A, Weissberg I, Biton IE, Pienik R, Wang E, Kelly S, Alroy J, Raas-Rothschild A, Friedman A, Brügger B, Merrill AH, ... Futerman AH, et al. Encephalopathy caused by ablation of very long acyl chain ceramide synthesis may be largely due to reduced galactosylceramide levels. The Journal of Biological Chemistry. 286: 30022-33. PMID 21705317 DOI: 10.1074/Jbc.M111.261206 |
0.306 |
|
| 2011 |
Shachar T, Lo Bianco C, Recchia A, Wiessner C, Raas-Rothschild A, Futerman AH. Lysosomal storage disorders and Parkinson's disease: Gaucher disease and beyond. Movement Disorders : Official Journal of the Movement Disorder Society. 26: 1593-604. PMID 21618611 DOI: 10.1002/Mds.23774 |
0.383 |
|
| 2011 |
Goldschmidt-Arzi M, Shimoni E, Sabanay H, Futerman AH, Addadi L. Intracellular localization of organized lipid domains of C16-ceramide/cholesterol. Journal of Structural Biology. 175: 21-30. PMID 21473916 DOI: 10.1016/J.Jsb.2011.03.021 |
0.311 |
|
| 2011 |
Farfel-Becker T, Vitner EB, Pressey SN, Eilam R, Cooper JD, Futerman AH. Spatial and temporal correlation between neuron loss and neuroinflammation in a mouse model of neuronopathic Gaucher disease. Human Molecular Genetics. 20: 1375-86. PMID 21252206 DOI: 10.1093/Hmg/Ddr019 |
0.362 |
|
| 2010 |
Offman MN, Krol M, Silman I, Sussman JL, Futerman AH. Molecular basis of reduced glucosylceramidase activity in the most common Gaucher disease mutant, N370S Journal of Biological Chemistry. 285: 42105-42114. PMID 20980259 DOI: 10.1074/Jbc.M110.172098 |
0.344 |
|
| 2010 |
Stiban J, Tidhar R, Futerman AH. Ceramide synthases: roles in cell physiology and signaling. Advances in Experimental Medicine and Biology. 688: 60-71. PMID 20919646 DOI: 10.1007/978-1-4419-6741-1_4 |
0.376 |
|
| 2010 |
Vitner EB, Dekel H, Zigdon H, Shachar T, Farfel-Becker T, Eilam R, Karlsson S, Futerman AH. Altered expression and distribution of cathepsins in neuronopathic forms of Gaucher disease and in other sphingolipidoses. Human Molecular Genetics. 19: 3583-90. PMID 20616152 DOI: 10.1093/Hmg/Ddq273 |
0.368 |
|
| 2010 |
Ben-David O, Futerman AH. The role of the ceramide acyl chain length in neurodegeneration: involvement of ceramide synthases. Neuromolecular Medicine. 12: 341-50. PMID 20502986 DOI: 10.1007/S12017-010-8114-X |
0.362 |
|
| 2010 |
Vitner EB, Platt FM, Futerman AH. Common and uncommon pathogenic cascades in lysosomal storage diseases. The Journal of Biological Chemistry. 285: 20423-7. PMID 20430897 DOI: 10.1074/Jbc.R110.134452 |
0.384 |
|
| 2010 |
Levy M, Futerman AH. Mammalian ceramide synthases. Iubmb Life. 62: 347-56. PMID 20222015 DOI: 10.1002/Iub.319 |
0.348 |
|
| 2010 |
Pewzner-Jung Y, Brenner O, Braun S, Laviad EL, Ben-Dor S, Feldmesser E, Horn-Saban S, Amann-Zalcenstein D, Raanan C, Berkutzki T, Erez-Roman R, Ben-David O, Levy M, Holzman D, Park H, ... ... Futerman AH, et al. A critical role for ceramide synthase 2 in liver homeostasis: II. insights into molecular changes leading to hepatopathy. The Journal of Biological Chemistry. 285: 10911-23. PMID 20110366 DOI: 10.1074/Jbc.M109.077610 |
0.33 |
|
| 2010 |
Pewzner-Jung Y, Park H, Laviad EL, Silva LC, Lahiri S, Stiban J, Erez-Roman R, Brügger B, Sachsenheimer T, Wieland F, Prieto M, Merrill AH, Futerman AH. A critical role for ceramide synthase 2 in liver homeostasis: I. alterations in lipid metabolic pathways. The Journal of Biological Chemistry. 285: 10902-10. PMID 20110363 DOI: 10.1074/Jbc.M109.077594 |
0.367 |
|
| 2010 |
Sridevi P, Alexander H, Laviad EL, Min J, Mesika A, Hannink M, Futerman AH, Alexander S. Stress-induced ER to Golgi translocation of ceramide synthase 1 is dependent on proteasomal processing. Experimental Cell Research. 316: 78-91. PMID 19800881 DOI: 10.1016/J.Yexcr.2009.09.027 |
0.313 |
|
| 2010 |
Brumshtein B, Salinas P, Peterson B, Chan V, Silman I, Sussman JL, Savickas PJ, Robinson GS, Futerman AH. Characterization of gene-activated human acid-β-glucosidase: Crystal structure, glycan composition, and internalization into macrophages Glycobiology. 20: 24-32. PMID 19741058 DOI: 10.1093/Glycob/Cwp138 |
0.353 |
|
| 2010 |
Farfel-Becker T, Futerman AH. Cellular pathogenesis in sphingolipid storage disorders: The quest for new therapeutic approaches Clinical Lipidology. 5: 255-265. DOI: 10.2217/Clp.10.13 |
0.347 |
|
| 2009 |
Goldfinger M, Laviad EL, Hadar R, Shmuel M, Dagan A, Park H, Merrill AH, Ringel I, Futerman AH, Tirosh B. De novo ceramide synthesis is required for N-linked glycosylation in plasma cells. Journal of Immunology (Baltimore, Md. : 1950). 182: 7038-47. PMID 19454701 DOI: 10.4049/Jimmunol.0802990 |
0.325 |
|
| 2009 |
Sridevi P, Alexander H, Laviad EL, Pewzner-Jung Y, Hannink M, Futerman AH, Alexander S. Ceramide synthase 1 is regulated by proteasomal mediated turnover. Biochimica Et Biophysica Acta. 1793: 1218-27. PMID 19393694 DOI: 10.1016/J.Bbamcr.2009.04.006 |
0.334 |
|
| 2009 |
Silva LC, Futerman AH, Prieto M. Lipid raft composition modulates sphingomyelinase activity and ceramide-induced membrane physical alterations. Biophysical Journal. 96: 3210-22. PMID 19383465 DOI: 10.1016/J.Bpj.2008.12.3923 |
0.316 |
|
| 2009 |
Kacher Y, Futerman AH. Impaired IL-10 transcription and release in animal models of Gaucher disease macrophages. Blood Cells, Molecules & Diseases. 43: 134-7. PMID 19380242 DOI: 10.1016/J.Bcmd.2009.03.006 |
0.742 |
|
| 2009 |
Lahiri S, Park H, Laviad EL, Lu X, Bittman R, Futerman AH. Ceramide synthesis is modulated by the sphingosine analog FTY720 via a mixture of uncompetitive and noncompetitive inhibition in an Acyl-CoA chain length-dependent manner. The Journal of Biological Chemistry. 284: 16090-8. PMID 19357080 DOI: 10.1074/Jbc.M807438200 |
0.331 |
|
| 2009 |
Farfel-Becker T, Vitner E, Dekel H, Leshem N, Enquist IB, Karlsson S, Futerman AH. No evidence for activation of the unfolded protein response in neuronopathic models of Gaucher disease. Human Molecular Genetics. 18: 1482-8. PMID 19193629 DOI: 10.1093/Hmg/Ddp061 |
0.396 |
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| 2008 |
Kacher Y, Brumshtein B, Boldin-Adamsky S, Toker L, Shainskaya A, Silman I, Sussman JL, Futerman AH. Acid β-glucosidase: Insights from structural analysis and relevance to Gaucher disease therapy Biological Chemistry. 389: 1361-1369. PMID 18783340 DOI: 10.1515/Bc.2008.163 |
0.771 |
|
| 2008 |
Laviad EL, Albee L, Pankova-Kholmyansky I, Epstein S, Park H, Merrill AH, Futerman AH. Characterization of ceramide synthase 2: tissue distribution, substrate specificity, and inhibition by sphingosine 1-phosphate. The Journal of Biological Chemistry. 283: 5677-84. PMID 18165233 DOI: 10.1074/Jbc.M707386200 |
0.362 |
|
| 2007 |
Brumshtein B, Greenblatt HM, Butters TD, Shaaltiel Y, Aviezer D, Silman I, Futerman AH, Sussman JL. Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid β-glucosidase: Insights into the mechanism of chemical chaperone action in Gaucher disease Journal of Biological Chemistry. 282: 29052-29058. PMID 17666401 DOI: 10.1074/Jbc.M705005200 |
0.336 |
|
| 2007 |
Lahiri S, Futerman AH. The metabolism and function of sphingolipids and glycosphingolipids Cellular and Molecular Life Sciences. 64: 2270-2284. PMID 17558466 DOI: 10.1007/S00018-007-7076-0 |
0.379 |
|
| 2007 |
Shaaltiel Y, Bartfeld D, Hashmueli S, Baum G, Brill-Almon E, Galili G, Dym O, Boldin-Adamsky SA, Silman I, Sussman JL, Futerman AH, Aviezer D. Production of glucocerebrosidase with terminal mannose glycans for enzyme replacement therapy of Gaucher's disease using a plant cell system. Plant Biotechnology Journal. 5: 579-90. PMID 17524049 DOI: 10.1111/J.1467-7652.2007.00263.X |
0.367 |
|
| 2007 |
Kacher Y, Golan A, Pewzner-Jung Y, Futerman AH. Changes in macrophage morphology in a Gaucher disease model are dependent on CTP:phosphocholine cytidylyltransferase alpha. Blood Cells, Molecules & Diseases. 39: 124-9. PMID 17482853 DOI: 10.1016/J.Bcmd.2007.03.005 |
0.76 |
|
| 2006 |
Brumshtein B, Wormald MR, Silman I, Futerman AH, Sussman JL. Structural comparison of differently glycosylated forms of acid-β-glucosidase, the defective enzyme in Gaucher disease Acta Crystallographica Section D: Biological Crystallography. 62: 1458-1465. PMID 17139081 DOI: 10.1107/S0907444906038303 |
0.33 |
|
| 2006 |
Futerman AH. Intracellular trafficking of sphingolipids: relationship to biosynthesis. Biochimica Et Biophysica Acta. 1758: 1885-92. PMID 16996025 DOI: 10.1016/J.Bbamem.2006.08.004 |
0.317 |
|
| 2006 |
Kacher Y, Futerman AH. Genetic diseases of sphingolipid metabolism: pathological mechanisms and therapeutic options. Febs Letters. 580: 5510-7. PMID 16970941 DOI: 10.1016/J.Febslet.2006.08.041 |
0.777 |
|
| 2006 |
Pewzner-Jung Y, Ben-Dor S, Futerman AH. When do Lasses (longevity assurance genes) become CerS (ceramide synthases)?: Insights into the regulation of ceramide synthesis. The Journal of Biological Chemistry. 281: 25001-5. PMID 16793762 DOI: 10.1074/Jbc.R600010200 |
0.303 |
|
| 2006 |
Kacher Y, Futerman AH. Neuronal cell death in glycosphingolipidoses Sphingolipid Biology. 285-293. DOI: 10.1007/4-431-34200-1_22 |
0.776 |
|
| 2006 |
Futerman AH. Sphingolipid and Glycolipid Biology Reviews in Cell Biology and Molecular Medicine. DOI: 10.1002/3527600906.Mcb.200400160 |
0.391 |
|
| 2005 |
Ginzburg L, Futerman AH. Defective calcium homeostasis in the cerebellum in a mouse model of Niemann-Pick A disease. Journal of Neurochemistry. 95: 1619-28. PMID 16277603 DOI: 10.1111/J.1471-4159.2005.03534.X |
0.319 |
|
| 2005 |
Lahiri S, Futerman AH. LASS5 is a bona fide dihydroceramide synthase that selectively utilizes palmitoyl-CoA as acyl donor. The Journal of Biological Chemistry. 280: 33735-8. PMID 16100120 DOI: 10.1074/Jbc.M506485200 |
0.313 |
|
| 2005 |
Futerman AH, Riezman H. The ins and outs of sphingolipid synthesis. Trends in Cell Biology. 15: 312-8. PMID 15953549 DOI: 10.1016/J.Tcb.2005.04.006 |
0.357 |
|
| 2005 |
Premkumar L, Sawkar AR, Boldin-Adamsky S, Toker L, Silman I, Kelly JW, Futerman AH, Sussman JL. X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease. The Journal of Biological Chemistry. 280: 23815-9. PMID 15817452 DOI: 10.1074/Jbc.M502799200 |
0.318 |
|
| 2005 |
Jmoudiak M, Futerman AH. Gaucher disease: pathological mechanisms and modern management. British Journal of Haematology. 129: 178-88. PMID 15813845 DOI: 10.1111/J.1365-2141.2004.05351.X |
0.392 |
|
| 2005 |
Cohen T, Auerbach W, Ravid L, Bodennec J, Fein A, Futerman AH, Joyner AL, Horowitz M. The exon 8-containing prosaposin gene splice variant is dispensable for mouse development, lysosomal function, and secretion. Molecular and Cellular Biology. 25: 2431-40. PMID 15743835 DOI: 10.1128/Mcb.25.6.2431-2440.2005 |
0.315 |
|
| 2005 |
Pelled D, Trajkovic-Bodennec S, Lloyd-Evans E, Sidransky E, Schiffmann R, Futerman AH. Enhanced calcium release in the acute neuronopathic form of Gaucher disease. Neurobiology of Disease. 18: 83-8. PMID 15649698 DOI: 10.1016/J.Nbd.2004.09.004 |
0.361 |
|
| 2004 |
Buccoliero R, Ginzburg L, Futerman AH. Elevation of lung surfactant phosphatidylcholine in mouse models of Sandhoff and of Niemann-Pick A disease Journal of Inherited Metabolic Disease. 27: 641-648. PMID 15669680 DOI: 10.1023/B:Boli.0000042958.22066.6C |
0.356 |
|
| 2004 |
Raas-Rothschild A, Pankova-Kholmyansky I, Kacher Y, Futerman AH. Glycosphingolipidoses: beyond the enzymatic defect. Glycoconjugate Journal. 21: 295-304. PMID 15514478 DOI: 10.1023/B:Glyc.0000046272.38480.Ef |
0.773 |
|
| 2004 |
Futerman AH, Hannun YA. The complex life of simple sphingolipids. Embo Reports. 5: 777-82. PMID 15289826 DOI: 10.1038/Sj.Embor.7400208 |
0.331 |
|
| 2004 |
Futerman AH, Van Meer G. The cell biology of lysosomal storage disorders Nature Reviews Molecular Cell Biology. 5: 554-565. PMID 15232573 DOI: 10.1038/Nrm1423 |
0.35 |
|
| 2004 |
Trajkovic-Bodennec S, Bodennec J, Futerman AH. Phosphatidylcholine metabolism is altered in a monocyte-derived macrophage model of Gaucher disease but not in lymphocytes Blood Cells, Molecules, and Diseases. 33: 77-82. PMID 15223015 DOI: 10.1016/J.Bcmd.2004.03.001 |
0.384 |
|
| 2004 |
Ginzburg L, Kacher Y, Futerman AH. The pathogenesis of glycosphingolipid storage disorders. Seminars in Cell & Developmental Biology. 15: 417-31. PMID 15207832 DOI: 10.1016/J.Semcdb.2004.03.003 |
0.764 |
|
| 2004 |
Buccoliero R, Bodennec J, Van Echten-Deckert G, Sandhoff K, Futerman AH. Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease Journal of Neurochemistry. 90: 80-88. PMID 15198669 DOI: 10.1111/J.1471-4159.2004.02457.X |
0.386 |
|
| 2004 |
Futerman AH, Sussman JL, Horowitz M, Silman I, Zimran A. New directions in the treatment of Gaucher disease. Trends in Pharmacological Sciences. 25: 147-51. PMID 15019270 DOI: 10.1016/J.Tips.2004.01.004 |
0.384 |
|
| 2003 |
Riebeling C, Allegood JC, Wang E, Merrill AH, Futerman AH. Two Mammalian Longevity Assurance Gene (LAG1) Family Members, trh1 and trh4, Regulate Dihydroceramide Synthesis Using Different Fatty Acyl-CoA Donors Journal of Biological Chemistry. 278: 43452-43459. PMID 12912983 DOI: 10.1074/Jbc.M307104200 |
0.32 |
|
| 2003 |
Dvir H, Harel M, McCarthy AA, Toker L, Silman I, Futerman AH, Sussman JL. X-ray structure of human acid-beta-glucosidase, the defective enzyme in Gaucher disease. Embo Reports. 4: 704-9. PMID 12792654 DOI: 10.1038/Sj.Embor.Embor873 |
0.331 |
|
| 2003 |
Pelled D, Lloyd-Evans E, Riebeling C, Jeyakumar M, Platt FM, Futerman AH. Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin Journal of Biological Chemistry. 278: 29496-29501. PMID 12756243 DOI: 10.1074/Jbc.M302964200 |
0.382 |
|
| 2003 |
Buccoliero R, Futerman AH. The roles of ceramide and complex sphingolipids in neuronal cell function Pharmacological Research. 47: 409-419. PMID 12676515 DOI: 10.1016/S1043-6618(03)00049-5 |
0.323 |
|
| 2002 |
Buccoliero R, Bodennec J, Futerman AH. The role of sphingolipids in neuronal development: Lessons from models of sphingolipid storage diseases Neurochemical Research. 27: 565-574. PMID 12374191 DOI: 10.1023/A:1020207613196 |
0.379 |
|
| 2002 |
Bodennec J, Pelled D, Riebeling C, Trajkovic S, Futerman AH. Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP:phosphocholine cytidylyltransferase by glucosylceramide The Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 16: 1814-1816. PMID 12223447 DOI: 10.1096/Fj.02-0149Fje |
0.38 |
|
| 2002 |
Venkataraman K, Riebeling C, Bodennec J, Riezman H, Allegood JC, Sullards MC, Merrill AH, Futerman AH. Upstream of growth and differentiation factor 1 (uog1), a mammalian homolog of the yeast longevity assurance gene 1 (LAG1), regulates N-stearoyl-sphinganine (C18-(dihydro)ceramide) synthesis in a fumonisin B1-independent manner in mammalian cells. The Journal of Biological Chemistry. 277: 35642-9. PMID 12105227 DOI: 10.1074/Jbc.M205211200 |
0.334 |
|
| 2002 |
Pelled D, Raveh T, Riebeling C, Fridkin M, Berissi H, Futerman AH, Kimchi A. Death-associated protein (DAP) kinase plays a central role in ceramide-induced apoptosis in cultured hippocampal neurons. The Journal of Biological Chemistry. 277: 1957-61. PMID 11709549 DOI: 10.1074/Jbc.M104677200 |
0.309 |
|
| 2001 |
Venkataraman K, Futerman AH. Comparison of the metabolism of L-erythro- and L-threo-sphinganines and ceramides in cultured cells and in subcellular fractions. Biochimica Et Biophysica Acta. 1530: 219-26. PMID 11239824 DOI: 10.1016/S1388-1981(01)00085-3 |
0.306 |
|
| 2000 |
Pelled D, Shogomori H, Futerman AH. The increased sensitivity of neurons with elevated glucocerebroside to neurotoxic agents can be reversed by imiglucerase Journal of Inherited Metabolic Disease. 23: 175-184. PMID 10801059 DOI: 10.1023/A:1005622001239 |
0.362 |
|
| 2000 |
Neuberger Y, Shogomori H, Levy Z, Fainzilber M, Futerman AH. A lyso-platelet activating factor phospholipase C, originally suggested to be a neutral-sphingomyelinase, is located in the endoplasmic reticulum. Febs Letters. 469: 44-6. PMID 10708753 DOI: 10.1016/S0014-5793(00)01235-7 |
0.312 |
|
| 1999 |
Korkotian E, Schwarz A, Pelled D, Schwarzmann G, Segal M, Futerman AH. Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons Journal of Biological Chemistry. 274: 21673-21678. PMID 10419477 DOI: 10.1074/Jbc.274.31.21673 |
0.343 |
|
| 1999 |
Naslavsky N, Shmeeda H, Friedlander G, Yanai A, Futerman AH, Barenholz Y, Taraboulos A. Sphingolipid depletion increases formation of the scrapie prion protein in neuroblastoma cells infected with prions Journal of Biological Chemistry. 274: 20763-20771. PMID 10409615 DOI: 10.1074/Jbc.274.30.20763 |
0.309 |
|
| 1999 |
Meivar-Levy I, Futerman AH. Up-regulation of neutral glycosphingolipid synthesis upon long term inhibition of ceramide synthesis by fumonisin B1 Journal of Biological Chemistry. 274: 4607-4612. PMID 9988695 DOI: 10.1074/Jbc.274.8.4607 |
0.307 |
|
| 1998 |
Futerman AH, Boldin S, Brann AB, Schwarz A, Zisling R. Regulatory roles for sphingolipids in the growth of polarized neurons Annals of the New York Academy of Sciences. 845: 176-187. PMID 9668351 DOI: 10.1111/J.1749-6632.1998.Tb09670.X |
0.302 |
|
| 1998 |
Van Echten-Deckert G, Giannis A, Schwarz A, Futerman AH, Sandhoff K. 1-Methylthiodihydroceramide, a novel analog of dihydroceramide, stimulates sphinganine degradation resulting in decreased de novo sphingolipid biosynthesis Journal of Biological Chemistry. 273: 1184-1191. PMID 9422785 DOI: 10.1074/Jbc.273.2.1184 |
0.332 |
|
| 1997 |
Schwarz A, Futerman AH. Distinct roles for ceramide and glucosylceramide at different stages of neuronal growth Journal of Neuroscience. 17: 2929-2938. PMID 9096129 DOI: 10.1523/Jneurosci.17-09-02929.1997 |
0.319 |
|
| 1997 |
Meivar-Levy I, Sabanay H, Bershadsky AD, Futerman AH. The role of sphingolipids in the maintenance of fibroblast morphology. The inhibition of protrusional activity, cell spreading, and cytokinesis induced by fumonisin B1 can be reversed by ganglioside GM3. The Journal of Biological Chemistry. 272: 1558-64. PMID 8999828 DOI: 10.1074/Jbc.272.3.1558 |
0.309 |
|
| 1994 |
Meivar-Levy I, Horowitz M, Futerman AH. Analysis of glucocerebrosidase activity using N-(1-[14C]hexanoyl)-D-erythro-glucosylsphingosine demonstrates a correlation between levels of residual enzyme activity and the type of Gaucher disease Biochemical Journal. 303: 377-382. PMID 7980395 DOI: 10.1042/Bj3030377 |
0.369 |
|
| 1993 |
Hirschberg K, Rodger J, Futerman AH. The long-chain sphingoid base of sphingolipids is acylated at the cytosolic surface of the endoplasmic reticulum in rat liver Biochemical Journal. 290: 751-757. PMID 8457204 DOI: 10.1042/Bj2900751 |
0.311 |
|
| 1992 |
Futerman AH, Pagano RE. Use of N-([1-14C]Hexanoyl)-D-erythro-sphingolipids to assay sphingolipid metabolism Methods in Enzymology. 209: 437-446. PMID 1495424 DOI: 10.1016/0076-6879(92)09054-7 |
0.325 |
|
| 1987 |
Silman I, Futerman AH. Posttranslational modification as a means of anchoring acetylcholinesterase to the cell surface Biopolymers - Peptide Science Section. 26. PMID 3555636 DOI: 10.1002/Bip.360260021 |
0.301 |
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