{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T14:20:14Z","timestamp":1782224414105,"version":"3.54.5"},"reference-count":37,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2015,7,26]],"date-time":"2015-07-26T00:00:00Z","timestamp":1437868800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Metab Brain Dis"],"published-print":{"date-parts":[[2015,10]]},"DOI":"10.1007\/s11011-015-9705-x","type":"journal-article","created":{"date-parts":[[2015,7,24]],"date-time":"2015-07-24T20:26:16Z","timestamp":1437769576000},"page":"1257-1267","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Activities of genes controlling sphingolipid metabolism in human fibroblasts treated with flavonoids"],"prefix":"10.1007","volume":"30","author":[{"given":"Marta","family":"Moskot","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Jak\u00f3bkiewicz-Banecka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elwira","family":"Smoli\u0144ska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bogdan","family":"Banecki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grzegorz","family":"W\u0119grzyn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2664-8724","authenticated-orcid":false,"given":"Magdalena","family":"Gabig-Cimi\u0144ska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2015,7,26]]},"reference":[{"key":"9705_CR1","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1007\/s10545-009-9029-2","volume":"33","author":"A Arfi","year":"2010","unstructured":"Arfi A, Richard M, Gandolphe C, Scherman D (2010) Storage correction in cells of patients suffering from mucopolysaccharidoses types IIIA and VII after treatment with genistein and other isoflavones. J Inherit Metab Dis 33:61\u201367","journal-title":"J Inherit Metab Dis"},{"key":"9705_CR2","doi-asserted-by":"crossref","first-page":"77","DOI":"10.7326\/0003-4819-146-2-200701160-00148","volume":"146","author":"M Banikazemi","year":"2007","unstructured":"Banikazemi M, Bultas J, Waldek S, Wilcox WR, Whitley CB, Mcdonald M, Finkel R, Packman S, Bichet DG, Warnock DG, Desnick RJ (2007) Agalsidase-beta therapy for advanced Fabry disease: a randomized trial. Ann Intern Med 146:77\u201386","journal-title":"Ann Intern Med"},{"key":"9705_CR3","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.ajhg.2013.05.006","volume":"93","author":"A Boukhris","year":"2013","unstructured":"Boukhris A, Schule R, Loureiro JL, Lourenco CM, Mundwiller E, Gonzalez MA, Charles P, Gauthier J, Rekik I, Acosta Lebrigio RF, Gaussen M, Speziani F, Ferbert A, Feki I, Caballero-Oteyza A, Dionne-Laporte A, Amri M, Noreau A, Forlani S, Cruz VT, Mochel F, Coutinho P, Dion P, Mhiri C, Schols L, Pouget J, Darios F, Rouleau GA, Marques Jr W, Brice A, Durr A, Zuchner S, Stevanin G (2013) Alteration of ganglioside biosynthesis responsible for complex hereditary spastic paraplegia. Am J Hum Genet 93:118\u2013123","journal-title":"Am J Hum Genet"},{"key":"9705_CR4","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1146\/annurev.med.57.110104.115650","volume":"57","author":"RO Brady","year":"2006","unstructured":"Brady RO (2006) Enzyme replacement for lysosomal diseases. Annu Rev Med 57:283\u2013296","journal-title":"Annu Rev Med"},{"key":"9705_CR5","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1159\/000112332","volume":"6","author":"E Conzelmann","year":"1983","unstructured":"Conzelmann E, Sandhoff K (1983) Partial enzyme deficiencies: residual activities and the development of neurological disorders. Dev Neurosci 6:58\u201371","journal-title":"Dev Neurosci"},{"key":"9705_CR6","first-page":"1343","volume":"56","author":"K Ferlinz","year":"1995","unstructured":"Ferlinz K, Hurwitz R, Weiler M, Suzuki K, Sandhoff K, Vanier MT (1995) Molecular analysis of the acid sphingomyelinase deficiency in a family with an intermediate form of Niemann-Pick disease. Am J Hum Genet 56:1343\u20131349","journal-title":"Am J Hum Genet"},{"key":"9705_CR7","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1080\/08035320510028210","volume":"94","author":"V Gieselmann","year":"2005","unstructured":"Gieselmann V (2005) What can cell biology tell us about heterogeneity in lysosomal storage diseases? Acta Paediatr Suppl 94:80\u201386 discussion 79","journal-title":"Acta Paediatr Suppl"},{"key":"9705_CR8","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1093\/glycob\/8.9.885","volume":"8","author":"BK Gillard","year":"1998","unstructured":"Gillard BK, Clement RG, Marcus DM (1998) Variations among cell lines in the synthesis of sphingolipids in de novo and recycling pathways. Glycobiology 8:885\u2013890","journal-title":"Glycobiology"},{"key":"9705_CR9","doi-asserted-by":"crossref","first-page":"2641","DOI":"10.1517\/14656560903270520","volume":"10","author":"CE Hollak","year":"2009","unstructured":"Hollak CE, De Fost M, Van Dussen L, Vom Dahl S, Aerts JM (2009) Enzyme therapy for the treatment of type 1 gaucher disease: clinical outcomes and dose - response relationships. Expert Opin Pharmacother 10:2641\u20132652","journal-title":"Expert Opin Pharmacother"},{"key":"9705_CR10","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1186\/1423-0127-16-26","volume":"16","author":"J Jakobkiewicz-Banecka","year":"2009","unstructured":"Jakobkiewicz-Banecka J, Piotrowska E, Narajczyk M, Baranska S, Wegrzyn G (2009) Genistein-mediated inhibition of glycosaminoglycan synthesis, which corrects storage in cells of patients suffering from mucopolysaccharidoses, acts by influencing an epidermal growth factor-dependent pathway. J Biomed Sci 16:26","journal-title":"J Biomed Sci"},{"key":"9705_CR11","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.nbd.2009.01.015","volume":"34","author":"M Jeyakumar","year":"2009","unstructured":"Jeyakumar M, Williams I, Smith D, Cox TM, Platt FM (2009) Critical role of iron in the pathogenesis of the murine gangliosidoses. Neurobiol Dis 34:406\u2013416","journal-title":"Neurobiol Dis"},{"key":"9705_CR12","doi-asserted-by":"crossref","first-page":"D354","DOI":"10.1093\/nar\/gkj102","volume":"34","author":"M Kanehisa","year":"2006","unstructured":"Kanehisa M, Goto S, Hattori M, Aoki-Kinoshita KF, Itoh M, Kawashima S, Katayama T, Araki M, Hirakawa M (2006) From genomics to chemical genomics: new developments in KEGG. Nucleic Acids Res 34:D354\u2013D357","journal-title":"Nucleic Acids Res"},{"key":"9705_CR13","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s11011-011-9233-2","volume":"26","author":"A Kloska","year":"2011","unstructured":"Kloska A, Jakobkiewicz-Banecka J, Narajczyk M, Banecka-Majkutewicz Z, Wegrzyn G (2011) Effects of flavonoids on glycosaminoglycan synthesis: implications for substrate reduction therapy in Sanfilippo disease and other mucopolysaccharidoses. Metab Brain Dis 26:1\u20138","journal-title":"Metab Brain Dis"},{"key":"9705_CR14","doi-asserted-by":"crossref","first-page":"2057","DOI":"10.1016\/j.bbamem.2006.05.027","volume":"1758","author":"T Kolter","year":"2006","unstructured":"Kolter T, Sandhoff K (2006) Sphingolipid metabolism diseases. Biochim Biophys Acta 1758:2057\u20132079","journal-title":"Biochim Biophys Acta"},{"key":"9705_CR15","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1007\/BF00219337","volume":"88","author":"P Leinekugel","year":"1992","unstructured":"Leinekugel P, Michel S, Conzelmann E, Sandhoff K (1992) Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease. Hum Genet 88:513\u2013523","journal-title":"Hum Genet"},{"key":"9705_CR16","doi-asserted-by":"crossref","first-page":"448","DOI":"10.3928\/00904481-20090723-09","volume":"38","author":"ER Lim-Melia","year":"2009","unstructured":"Lim-Melia ER, Kronn DF (2009) Current enzyme replacement therapy for the treatment of lysosomal storage diseases. Pediatr Ann 38:448\u2013455","journal-title":"Pediatr Ann"},{"key":"9705_CR17","doi-asserted-by":"crossref","first-page":"17054","DOI":"10.1074\/jbc.M114.555300","volume":"289","author":"M Moskot","year":"2014","unstructured":"Moskot M, Montefusco S, Jakobkiewicz-Banecka J, Mozolewski P, Wegrzyn A, Di Bernardo D, Wegrzyn G, Medina DL, Ballabio A, Gabig-Ciminska M (2014) The phytoestrogen genistein modulates lysosomal metabolism and transcription factor EB (TFEB) activation. J Biol Chem 289:17054\u201317069","journal-title":"J Biol Chem"},{"key":"9705_CR18","doi-asserted-by":"crossref","first-page":"9378","DOI":"10.1038\/srep09378","volume":"5","author":"M Moskot","year":"2015","unstructured":"Moskot M, Jakobkiewicz-Banecka J, Kloska A, Smolinska E, Mozolewski P, Malinowska M, Rychlowski M, Banecki B, Wegrzyn G, Gabig-Ciminska M (2015) Modulation of expression of genes involved in glycosaminoglycan metabolism and lysosome biogenesis by flavonoids. Sci Rep 5:9378","journal-title":"Sci Rep"},{"key":"9705_CR19","doi-asserted-by":"crossref","first-page":"41","DOI":"10.2165\/11318540-000000000-00000","volume":"24","author":"GM Pastores","year":"2010","unstructured":"Pastores GM (2010) Recombinant glucocerebrosidase (imiglucerase) as a therapy for Gaucher disease. BioDrugs 24:41\u201347","journal-title":"BioDrugs"},{"key":"9705_CR20","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1016\/j.clinthera.2005.08.004","volume":"27","author":"GM Pastores","year":"2005","unstructured":"Pastores GM, Barnett NL, Kolodny EH (2005) An open-label, noncomparative study of miglustat in type I Gaucher disease: efficacy and tolerability over 24\u00a0months of treatment. Clin Ther 27:1215\u20131227","journal-title":"Clin Ther"},{"key":"9705_CR21","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1038\/sj.ejhg.5201623","volume":"14","author":"E Piotrowska","year":"2006","unstructured":"Piotrowska E, Jakobkiewicz-Banecka J, Baranska S, Tylki-Szymanska A, Czartoryska B, Wegrzyn A, Wegrzyn G (2006) Genistein-mediated inhibition of glycosaminoglycan synthesis as a basis for gene expression-targeted isoflavone therapy for mucopolysaccharidoses. Eur J Hum Genet 14:846\u2013852","journal-title":"Eur J Hum Genet"},{"key":"9705_CR22","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1083\/jcb.201208152","volume":"199","author":"FM Platt","year":"2012","unstructured":"Platt FM, Boland B, Van Der Spoel AC (2012) The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction. J Cell Biol 199:723\u2013734","journal-title":"J Cell Biol"},{"key":"9705_CR23","doi-asserted-by":"crossref","first-page":"2697","DOI":"10.2165\/00003495-200767180-00005","volume":"67","author":"M Rohrbach","year":"2007","unstructured":"Rohrbach M, Clarke JT (2007) Treatment of lysosomal storage disorders : progress with enzyme replacement therapy. Drugs 67:2697\u20132716","journal-title":"Drugs"},{"key":"9705_CR24","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1111\/j.1471-4159.2010.06976.x","volume":"116","author":"AI Rosenbaum","year":"2011","unstructured":"Rosenbaum AI, Maxfield FR (2011) Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches. J Neurochem 116:789\u2013795","journal-title":"J Neurochem"},{"key":"9705_CR25","doi-asserted-by":"crossref","first-page":"1562","DOI":"10.1042\/BST20130083","volume":"41","author":"K Sandhoff","year":"2013","unstructured":"Sandhoff K (2013) Metabolic and cellular bases of sphingolipidoses. Biochem Soc Trans 41:1562\u20131568","journal-title":"Biochem Soc Trans"},{"key":"9705_CR26","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1126\/science.1174447","volume":"325","author":"M Sardiello","year":"2009","unstructured":"Sardiello M, Palmieri M, Di Ronza A, Medina DL, Valenza M, Gennarino VA, Di Malta C, Donaudy F, Embrione V, Polishchuk RS, Banfi S, Parenti G, Cattaneo E, Ballabio A (2009) A gene network regulating lysosomal biogenesis and function. Science 325:473\u2013477","journal-title":"Science"},{"key":"9705_CR27","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1093\/acprof:oso\/9780198525387.003.0004","volume-title":"Neuroglycobiology","author":"RL Schnaar","year":"2005","unstructured":"Schnaar RL (2005) In: Fukuda M, Rutishauser U, Schnaar RL, Yamaguchi Y (eds) Neuroglycobiology. Oxford University Press, Oxford, pp. 95\u2013113"},{"key":"9705_CR28","volume-title":"Essentials of glycobiology","author":"RL Schnaar","year":"2009","unstructured":"Schnaar RL (2009) Essentials of glycobiology. Cold Spring Harbour Laboratory Press, Cold Spring Harbour"},{"key":"9705_CR29","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1016\/j.tins.2011.05.006","volume":"34","author":"ML Schultz","year":"2011","unstructured":"Schultz ML, Tecedor L, Chang M, Davidson BL (2011) Clarifying lysosomal storage diseases. Trends Neurosci 34:401\u2013410","journal-title":"Trends Neurosci"},{"key":"9705_CR30","doi-asserted-by":"crossref","first-page":"1225","DOI":"10.1038\/ng1460","volume":"36","author":"MA Simpson","year":"2004","unstructured":"Simpson MA, Cross H, Proukakis C, Priestman DA, Neville DC, Reinkensmeier G, Wang H, Wiznitzer M, Gurtz K, Verganelaki A, Pryde A, Patton MA, Dwek RA, Butters TD, Platt FM, Crosby AH (2004) Infantile-onset symptomatic epilepsy syndrome caused by a homozygous loss-of-function mutation of GM3 synthase. Nat Genet 36:1225\u20131229","journal-title":"Nat Genet"},{"key":"9705_CR31","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/j.ajhg.2009.09.016","volume":"85","author":"Y Tajima","year":"2009","unstructured":"Tajima Y, Kawashima I, Tsukimura T, Sugawara K, Kuroda M, Suzuki T, Togawa T, Chiba Y, Jigami Y, Ohno K, Fukushige T, Kanekura T, Itoh K, Ohashi T, Sakuraba H (2009) Use of a modified alpha-N-acetylgalactosaminidase in the development of enzyme replacement therapy for Fabry disease. Am J Hum Genet 85:569\u2013580","journal-title":"Am J Hum Genet"},{"key":"9705_CR32","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1016\/j.bbrc.2007.12.187","volume":"367","author":"A Takamura","year":"2008","unstructured":"Takamura A, Higaki K, Kajimaki K, Otsuka S, Ninomiya H, Matsuda J, Ohno K, Suzuki Y, Nanba E (2008) Enhanced autophagy and mitochondrial aberrations in murine G(M1)-gangliosidosis. Biochem Biophys Res Commun 367:616\u2013622","journal-title":"Biochem Biophys Res Commun"},{"key":"9705_CR33","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.ymgme.2009.09.006","volume":"99","author":"MA Tan","year":"2010","unstructured":"Tan MA, Fuller M, Zabidi-Hussin ZA, Hopwood JJ, Meikle PJ (2010) Biochemical profiling to predict disease severity in metachromatic leukodystrophy. Mol Genet Metab 99:142\u2013148","journal-title":"Mol Genet Metab"},{"key":"9705_CR34","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1016\/S0300-9084(03)00047-6","volume":"85","author":"G Tettamanti","year":"2003","unstructured":"Tettamanti G, Bassi R, Viani P, Riboni L (2003) Salvage pathways in glycosphingolipid metabolism. Biochimie 85:423\u2013437","journal-title":"Biochimie"},{"key":"9705_CR35","doi-asserted-by":"crossref","unstructured":"Tomatsu S, Almeciga-Diaz CJ, Montano AM, Yabe H, Tanaka A, Dung VC, Giugliani R, Kubaski F, Mason RW, Yasuda E, Sawamoto K, Mackenzie W, Suzuki Y, Orii KE, Barrera LA, Sly WS, Orii T (2014) Therapies for the bone in mucopolysaccharidoses. Mol Genet Metab","DOI":"10.1016\/j.ymgme.2014.12.267"},{"key":"9705_CR36","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.chroma.2004.10.048","volume":"1073","author":"TH Tsai","year":"2005","unstructured":"Tsai TH (2005) Concurrent measurement of unbound genistein in the blood, brain and bile of anesthetized rats using microdialysis and its pharmacokinetic application. J Chromatogr A 1073:317\u2013322","journal-title":"J Chromatogr A"},{"key":"9705_CR37","doi-asserted-by":"crossref","first-page":"9142","DOI":"10.1073\/pnas.96.16.9142","volume":"96","author":"T Yamashita","year":"1999","unstructured":"Yamashita T, Wada R, Sasaki T, Deng C, Bierfreund U, Sandhoff K, Proia RL (1999) A vital role for glycosphingolipid synthesis during development and differentiation. Proc Natl Acad Sci U S A 96:9142\u20139147","journal-title":"Proc Natl Acad Sci U S A"}],"updated-by":[{"DOI":"10.1007\/s11011-017-9974-7","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2017,2,22]],"date-time":"2017-02-22T00:00:00Z","timestamp":1487721600000}}],"container-title":["Metabolic Brain Disease"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11011-015-9705-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s11011-015-9705-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11011-015-9705-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,8,28]],"date-time":"2019-08-28T14:12:23Z","timestamp":1567001543000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s11011-015-9705-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,7,26]]},"references-count":37,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2015,10]]}},"alternative-id":["9705"],"URL":"https:\/\/doi.org\/10.1007\/s11011-015-9705-x","relation":{"correction":[{"id-type":"doi","id":"10.1007\/s11011-017-9974-7","asserted-by":"object"}]},"ISSN":["0885-7490","1573-7365"],"issn-type":[{"value":"0885-7490","type":"print"},{"value":"1573-7365","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,7,26]]}}}