{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T13:06:16Z","timestamp":1792674376904,"version":"4.5.0"},"reference-count":27,"publisher":"Public Library of Science (PLoS)","issue":"12","license":[{"start":{"date-parts":[[2015,12,14]],"date-time":"2015-12-14T00:00:00Z","timestamp":1450051200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0144958","type":"journal-article","created":{"date-parts":[[2015,12,14]],"date-time":"2015-12-14T19:45:31Z","timestamp":1450122331000},"page":"e0144958","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":25,"title":["Distributions of Globotriaosylceramide Isoforms, and Globotriaosylsphingosine and Its Analogues in an \u03b1-Galactosidase A Knockout Mouse, a Model of Fabry Disease"],"prefix":"10.1371","volume":"10","author":[{"given":"Hideaki","family":"Sueoka","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikio","family":"Aoki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takahiro","family":"Tsukimura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tadayasu","family":"Togawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hitoshi","family":"Sakuraba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2015,12,14]]},"reference":[{"key":"ref1","first-page":"3733","article-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"RJ Desnick","year":"2001"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1186\/1750-1172-5-30","article-title":"Fabry disease","volume":"5","author":"DP Germain","year":"2010","journal-title":"Orphanet J Rare Dis"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"2812","DOI":"10.1073\/pnas.0712309105","article-title":"Elevated globotriaosylsphingosine is a hallmark of Fabry disease","volume":"105","author":"JM Aerts","year":"2008","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.ymgme.2010.03.020","article-title":"Plasma globotriaosylsphingosine as a biomarker of Fabry disease","volume":"100","author":"T Togawa","year":"2010","journal-title":"Mol Genet Metab"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1016\/j.bbadis.2010.05.003","article-title":"Plasma globotriaosylsphingosine: diagnostic value and relation to clinical manifestations of Fabry disease","volume":"1802","author":"SM Rombach","year":"2010","journal-title":"Biochim Biophys Acta"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.bbadis.2010.09.007","article-title":"Reduction of elevated plasma globotriaosylsphingosine in patients with classic Fabry disease following enzyme replacement therapy","volume":"1812","author":"MJ van Breemen","year":"2011","journal-title":"Biochim Biophys Acta"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"2745","DOI":"10.1021\/ac203433e","article-title":"Urinary globotriaosylsphingosine-related biomarkers for Fabry disease targeted by metabolomics","volume":"84","author":"C Auray-Blais","year":"2012","journal-title":"Anal Chem"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1373\/clinchem.2012.192138","article-title":"Quantification of globotriaosylsphingosine in plasma and urine of Fabry patients by stable isotope ultraperformance liquid chromatography-tandem mass spectrometry","volume":"59","author":"H Gold","year":"2013","journal-title":"Clin Chem"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1093\/cvr\/cvq333","article-title":"Globotriaosylceramide leads to K(Ca)3.1 channel dysfunction: a new insight into endothelial dysfunction in Fabry disease","volume":"89","author":"S Park","year":"2011","journal-title":"Cardiovasc Res"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1161\/ATVBAHA.113.302200","article-title":"Globotriaosylceramide induces lysosomal degradation of endothelial K<sub>Ca<\/sub>3.1 in Fabry disease","volume":"34","author":"S Choi","year":"2014","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1161\/ATVBAHA.113.302744","article-title":"Globotriaosylceramide induces endothelial dysfunction in Fabry disease","volume":"34","author":"K Satoh","year":"2014","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.3892\/ijmm.2012.1139","article-title":"Possible role of transforming growth factor-\u03b21 and vascular endothelial growth factor in Fabry disease nephropathy","volume":"30","author":"MH Lee","year":"2012","journal-title":"Int J Mol Med"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1093\/ndt\/gfq306","article-title":"Globotriaosylsphingosine actions on human glomerular podocytes: implications for Fabry nephropathy","volume":"26","author":"MD Sanchez-Ni\u00f1o","year":"2011","journal-title":"Nephrol Dial Transplant"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.neulet.2015.01.084","article-title":"The Fabry disease-associated lipid lyso-Gb3 enhances voltage-gated calcium currents in sensory neurons and causes pain","volume":"594","author":"L Choi","year":"2015","journal-title":"Neurosci Lett"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"2957","DOI":"10.1002\/rcm.4716","article-title":"Highly sensitive localization analysis of gangliosides and sulfatides including structural isomers in mouse cerebellum sections by combination of laser microdissection and hydrophilic interaction liquid chromatography\/electrospray ionization mass spectrometry with theoretically expanded multiple reaction monitoring","volume":"24","author":"K Ikeda","year":"2010","journal-title":"Rapid Commun Mass Spectrom"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"e0127048","DOI":"10.1371\/journal.pone.0127048","article-title":"Nano-LC-MS\/MS for quantification of lyso-Gb3 and its analogues reveals a useful biomarker for Fabry disease","volume":"10","author":"H Sueoka","year":"2015","journal-title":"PLoS One"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"2540","DOI":"10.1073\/pnas.94.6.2540","article-title":"\u03b1-Galactosidase A deficient mice: a model of Fabry disease","volume":"94","author":"T Ohshima","year":"1997","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref18","first-page":"A208","article-title":"Generation of a mouse model with \u03b1-galactosidase A deficiency","volume":"59","author":"AM Wang","year":"1996","journal-title":"Am J Hum Genet"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"7515","DOI":"10.1073\/pnas.120177997","article-title":"Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells","volume":"97","author":"T Takenaka","year":"2000","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/j.ajhg.2009.09.016","article-title":"Use of a modified alpha-N-acetylgalactosaminidase in the development of enzyme replacement therapy for Fabry disease","volume":"85","author":"Y Tajima","year":"2009","journal-title":"Am J Hum Genet"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1038\/mt.2009.220","article-title":"The pharmacological chaperone 1-deoxygalactonojirimycin reduces tissue globotriaosylceramide levels in a mouse model of Fabry disease","volume":"18","author":"R Khanna","year":"2010","journal-title":"Mol Ther"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"e15033","DOI":"10.1371\/journal.pone.0015033","article-title":"Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease","volume":"5","author":"J Marshall","year":"2010","journal-title":"PLoS One"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"2283","DOI":"10.1007\/s00216-014-8402-7","article-title":"Fabry disease: renal sphingolipid distribution in the \u03b1-Gal A knockout mouse model by mass spectrometric and immunohistochemical imaging","volume":"407","author":"L Kuchar","year":"2015","journal-title":"Anal Bioanal Chem"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.nbd.2008.09.001","article-title":"Neurophysiological, behavioral and morphological abnormalities in the Fabry knockout mice","volume":"33","author":"LG Rodrigues","year":"2009","journal-title":"Neurobiol Dis"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1016\/j.ajpath.2014.11.004","article-title":"\u03b1-Galactosidase A knockout mice: progressive organ pathology resembles the type 2 later-onset phenotype of Fabry disease","volume":"185","author":"DS Bangari","year":"2015","journal-title":"Am J Pathol"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"6423","DOI":"10.1073\/pnas.96.11.6423","article-title":"Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice","volume":"96","author":"T Ohshima","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.chroma.2013.01.078","article-title":"Development of a lipid profiling system using reverse-phase liquid chromatography coupled to high-resolution mass spectrometry with rapid polarity switching and an automated lipid identification software","volume":"1292","author":"T Yamada","year":"2013","journal-title":"J Chromatogr A"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0144958","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,9,2]],"date-time":"2019-09-02T13:34:09Z","timestamp":1567431249000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0144958"}},"subtitle":[],"editor":[{"given":"Israel","family":"Silman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2015,12,14]]},"references-count":27,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2015,12,14]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0144958","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,12,14]]}}}