{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T06:08:38Z","timestamp":1790662118919,"version":"4.1.0"},"reference-count":38,"publisher":"Public Library of Science (PLoS)","issue":"4","license":[{"start":{"date-parts":[[2012,4,30]],"date-time":"2012-04-30T00:00:00Z","timestamp":1335744000000},"content-version":"unspecified","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.0036373","type":"journal-article","created":{"date-parts":[[2012,4,30]],"date-time":"2012-04-30T16:55:57Z","timestamp":1335804957000},"page":"e36373","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":44,"title":["Globotriaosylsphingosine Accumulation and Not Alpha-Galactosidase-A Deficiency Causes Endothelial Dysfunction in Fabry Disease"],"prefix":"10.1371","volume":"7","author":[{"given":"Mehdi","family":"Namdar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Gebhard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael","family":"Studiger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Shi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pavani","family":"Mocharla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Schmied","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro","family":"Brugada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas F.","family":"L\u00fcscher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni G.","family":"Camici","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2012,4,30]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1016\/S0140-6736(08)61589-5","article-title":"Fabry&apos;s disease.","volume":"372","author":"YA Zarate","year":"2008","journal-title":"Lancet"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1111\/j.1440-1681.2008.04984.x","article-title":"Vascular dysfunction in the alpha-galactosidase A-knockout mouse is an endothelial cell-, plasma membrane-based defect.","volume":"35","author":"JL Park","year":"2008","journal-title":"Clin Exp Pharmacol Physiol"},{"key":"ref3","first-page":"357","article-title":"Coronary microvascular dysfunction in male patients with Anderson-Fabry disease and the effect of treatment with alpha galactosidase A. Heart","volume":"92","author":"PM Elliott","year":"2006"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1007\/s10545-005-0563-2","article-title":"Impaired myocardial perfusion reserve but preserved peripheral endothelial function in patients with Fabry disease.","volume":"28","author":"RJ Kalliokoski","year":"2005","journal-title":"J Inherit Metab Dis"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1097\/GIM.0b013e31802d8321","article-title":"Heterozygous Fabry women are not just carriers, but have a significant burden of disease and impaired quality of life.","volume":"9","author":"RY Wang","year":"2007","journal-title":"Genet Med"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1097\/01.ASN.0000043901.45141.D4","article-title":"Fabry disease in mice is associated with age-dependent susceptibility to vascular thrombosis.","volume":"14","author":"DT Eitzman","year":"2003","journal-title":"J Am Soc Nephrol"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s00395-009-0060-0","article-title":"PDGF-CC induces tissue factor expression: role of PDGF receptor alpha\/beta.","volume":"105","author":"C Gebhard","year":"2010","journal-title":"Basic Res Cardiol"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"5217","DOI":"10.1073\/pnas.0609656104","article-title":"Genetic deletion of p66(Shc) adaptor protein prevents hyperglycemia-induced endothelial dysfunction and oxidative stress.","volume":"104","author":"GG Camici","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1038\/ki.2008.576","article-title":"Downregulation of alpha-galactosidase A upregulates CD77: functional impact for Fabry nephropathy.","volume":"75","author":"T Thomaidis","year":"2009","journal-title":"Kidney Int"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1007\/PL00008196","article-title":"Fabry disease: ultrastructural lectin histochemical analyses of lysosomal deposits.","volume":"436","author":"A Kanda","year":"2000","journal-title":"Virchows Arch"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1007\/s00428-007-0468-6","article-title":"Cellular and tissue localization of globotriaosylceramide in Fabry disease.","volume":"451","author":"H Askari","year":"2007","journal-title":"Virchows Arch"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"1559","DOI":"10.1161\/01.STR.32.7.1559","article-title":"Enhanced endothelium-dependent vasodilation in Fabry disease.","volume":"32","author":"G Altarescu","year":"2001","journal-title":"Stroke"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.1161\/hc3801.096352","article-title":"Regional cerebral hyperperfusion and nitric oxide pathway dysregulation in Fabry disease: reversal by enzyme replacement therapy.","volume":"104","author":"DF Moore","year":"2001","journal-title":"Circulation"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1161\/01.CIR.0000154550.15963.80","article-title":"Alpha-galactosidase A deficiency accelerates atherosclerosis in mice with apolipoprotein E deficiency.","volume":"111","author":"PF Bodary","year":"2005","journal-title":"Circulation"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"1975","DOI":"10.1681\/ASN.2008111190","article-title":"Decreased nitric oxide bioavailability in a mouse model of Fabry disease.","volume":"20","author":"L Shu","year":"2009","journal-title":"J Am Soc Nephrol"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"3099","DOI":"10.1161\/01.CIR.103.25.3099","article-title":"Genetic deficiency of inducible nitric oxide synthase reduces atherosclerosis and lowers plasma lipid peroxides in apolipoprotein E-knockout mice.","volume":"103","author":"PJ Kuhlencordt","year":"2001","journal-title":"Circulation"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1097\/01.fjc.0000187174.67661.67","article-title":"Endothelium-dependent contractions occur in the aorta of wild-type and COX2\u2212\/\u2212 knockout but not COX1\u2212\/\u2212 knockout mice.","volume":"46","author":"EH Tang","year":"2005","journal-title":"J Cardiovasc Pharmacol"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1038\/sj.bjp.0706042","article-title":"Endothelium-dependent contractions in hypertension.","volume":"144","author":"PM Vanhoutte","year":"2005","journal-title":"Br J Pharmacol"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"H1133","DOI":"10.1152\/ajpheart.00929.2008","article-title":"Differential involvement of COX1 and COX2 in the vasculopathy associated with the alpha-galactosidase A-knockout mouse.","volume":"296","author":"JL Park","year":"2009","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1097\/00004872-200309000-00015","article-title":"Selective cyclo-oxygenase-2 inhibition with parecoxib acutely impairs endothelium-dependent vasodilatation in patients with essential hypertension.","volume":"21","author":"D Bulut","year":"2003","journal-title":"J Hypertens"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"e12","DOI":"10.1161\/01.RES.0000241051.83067.62","article-title":"Increased cyclooxygenase-2 expression and prostaglandin-mediated dilation in coronary arterioles of patients with diabetes mellitus.","volume":"99","author":"T Szerafin","year":"2006","journal-title":"Circ Res"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"1950","DOI":"10.1161\/CIRCULATIONAHA.105.602425","article-title":"Use of first- and second-generation cyclooxygenase-2-selective nonsteroidal antiinflammatory drugs and risk of acute myocardial infarction.","volume":"113","author":"F Andersohn","year":"2006","journal-title":"Circulation"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1038\/sj.bjp.0707190","article-title":"Calcium and reactive oxygen species increase in endothelial cells in response to releasers of endothelium-derived contracting factor.","volume":"151","author":"EH Tang","year":"2007","journal-title":"Br J Pharmacol"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1038\/sj.bjp.0704669","article-title":"Oxygen-derived free radicals mediate endothelium-dependent contractions to acetylcholine in aortas from spontaneously hypertensive rats.","volume":"136","author":"D Yang","year":"2002","journal-title":"Br J Pharmacol"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"B85","DOI":"10.1093\/gerona\/55.2.B85","article-title":"Effect of aging on aortic expression of the vascular cell adhesion molecule-1 and atherosclerosis in murine models of atherosclerosis.","volume":"55","author":"S Merat","year":"2000","journal-title":"J Gerontol A Biol Sci Med Sci"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1111\/j.1365-2796.2007.01862.x","article-title":"C-reactive protein, interleukin-6, secretory phospholipase A2 group IIA and intercellular adhesion molecule-1 in the prediction of late outcome events after acute coronary syndromes.","volume":"262","author":"M Hartford","year":"2007","journal-title":"J Intern Med"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1002\/1531-8249(200002)47:2<229::AID-ANA13>3.0.CO;2-T","article-title":"Profile of endothelial and leukocyte activation in Fabry patients.","volume":"47","author":"T DeGraba","year":"2000","journal-title":"Ann Neurol"},{"key":"ref28","first-page":"57","article-title":"Endothelial markers and homocysteine in patients with classic Fabry disease.","volume":"Paediatr","author":"K Demuth","year":"2002","journal-title":"Acta"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.ymgme.2008.06.016","article-title":"Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells.","volume":"95","author":"JS Shen","year":"2008","journal-title":"Mol Genet Metab"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1002\/ana.410400105","article-title":"Cerebrovascular complications of Fabry&apos;s disease.","volume":"40","author":"P Mitsias","year":"1996","journal-title":"Ann Neurol"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"1746","DOI":"10.1212\/WNL.50.6.1746","article-title":"Quantitative analysis of cerebral vasculopathy in patients with Fabry disease.","volume":"50","author":"KE Crutchfield","year":"1998","journal-title":"Neurology"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/BF00868342","article-title":"Stroke in Fabry&apos;s disease.","volume":"241","author":"RP Grewal","year":"1994","journal-title":"J Neurol"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.ymgme.2009.10.004","article-title":"Vasculopathy in patients with Fabry disease: current controversies and research directions.","volume":"99","author":"SM Rombach","year":"2010","journal-title":"Mol Genet Metab"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"338","DOI":"10.7326\/0003-4819-138-4-200302180-00014","article-title":"Fabry disease, an under-recognized multisystemic disorder: expert recommendations for diagnosis, management, and enzyme replacement therapy.","volume":"138","author":"RJ Desnick","year":"2003","journal-title":"Ann Intern Med"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1161\/01.ATV.0000209649.60409.38","article-title":"Cardiac and vascular hypertrophy in Fabry disease: evidence for a new mechanism independent of blood pressure and glycosphingolipid deposition.","volume":"26","author":"F Barbey","year":"2006","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"1222","DOI":"10.1177\/0091270007305299","article-title":"Enzyme replacement in Fabry disease: pharmacokinetics and pharmacodynamics of agalsidase alpha in children and adolescents.","volume":"47","author":"M Ries","year":"2007","journal-title":"J Clin Pharmacol"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1056\/NEJM200107053450102","article-title":"Safety and efficacy of recombinant human alpha-galactosidase A\u2013replacement therapy in Fabry&apos;s disease.","volume":"345","author":"CM Eng","year":"2001","journal-title":"N Engl J Med"},{"key":"ref38","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"}],"updated-by":[{"DOI":"10.1371\/annotation\/7b2c04df-8592-4fb7-8608-3039db28b504","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2012,9,12]],"date-time":"2012-09-12T00:00:00Z","timestamp":1347408000000}}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0036373","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,14]],"date-time":"2022-01-14T09:59:59Z","timestamp":1642154399000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0036373"}},"subtitle":[],"editor":[{"given":"Yu","family":"Huang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2012,4,30]]},"references-count":38,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2012,4,30]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0036373","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,4,30]]}}}