{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T17:02:41Z","timestamp":1771002161009,"version":"3.50.1"},"reference-count":36,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2012,10,1]],"date-time":"2012-10-01T00:00:00Z","timestamp":1349049600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["HD055461"],"award-info":[{"award-number":["HD055461"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100003874","name":"Hunter\u2019s Hope Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100003874","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100003189","name":"National Tay-Sachs and Allied Diseases Association","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100003189","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Experimental Neurology"],"published-print":{"date-parts":[[2012,10]]},"DOI":"10.1016\/j.expneurol.2012.07.013","type":"journal-article","created":{"date-parts":[[2012,7,28]],"date-time":"2012-07-28T16:34:44Z","timestamp":1343493284000},"page":"444-452","source":"Crossref","is-referenced-by-count":21,"title":["Oxidative stress as a therapeutic target in globoid cell leukodystrophy"],"prefix":"10.1016","volume":"237","author":[{"given":"Jacqueline A.","family":"Hawkins-Salsbury","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth Y.","family":"Qin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adarsh S.","family":"Reddy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carole A.","family":"Vogler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark S.","family":"Sands","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.expneurol.2012.07.013_bb0005","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1124\/jpet.109.157941","article-title":"Early intervention of tyrosine nitration prevents vaso-obliteration and neovascularization in ischemic retinopathy","volume":"332","author":"Abdelsaid","year":"2010","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0010","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.coph.2007.04.005","article-title":"N-acetylcysteine\u2014a safe antidote for cysteine\/glutathione deficiency","volume":"7","author":"Atkuri","year":"2007","journal-title":"Curr. Opin. Pharmacol."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0015","doi-asserted-by":"crossref","first-page":"424","DOI":"10.3109\/15563650.2010.486381","article-title":"A multicenter comparison of the safety of oral versus intravenous acetylcysteine for treatment of acetaminophen overdose","volume":"48","author":"Bebarta","year":"2010","journal-title":"Clin. Toxicol. (Phila.)"},{"key":"10.1016\/j.expneurol.2012.07.013_bb0020","doi-asserted-by":"crossref","first-page":"C1424","DOI":"10.1152\/ajpcell.1996.271.5.C1424","article-title":"Nitric oxide, superoxide, and peroxynitrite: the good, the bad, and ugly","volume":"271","author":"Beckman","year":"1996","journal-title":"Am. J. Physiol."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0025","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1203\/00006450-200201000-00009","article-title":"Substrate-reduction therapy enhances the benefits of bone marrow transplantation in young mice with globoid cell leukodystrophy","volume":"51","author":"Biswas","year":"2002","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0030","doi-asserted-by":"crossref","first-page":"98","DOI":"10.2353\/ajpath.2008.070404","article-title":"Clinical and immunopathologic alterations in rhesus macaques affected with globoid cell leukodystrophy","volume":"172","author":"Borda","year":"2008","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0035","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.abb.2008.06.012","article-title":"Psychosine-induced alterations in peroxisomes of twitcher mouse liver","volume":"477","author":"Contreras","year":"2008","journal-title":"Arch. Biochem. Biophys."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0040","doi-asserted-by":"crossref","first-page":"5952","DOI":"10.1158\/1078-0432.CCR-07-1187","article-title":"N-acetylcysteine protects melanocytes against oxidative stress\/damage and delays onset of ultraviolet-induced melanoma in mice","volume":"13","author":"Cotter","year":"2007","journal-title":"Clin. Cancer Res."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0045","doi-asserted-by":"crossref","first-page":"1173","DOI":"10.1046\/j.1471-4159.2003.01580.x","article-title":"The antioxidants alpha-lipoic acid and N-acetylcysteine reverse memory impairment and brain oxidative stress in aged SAMP8 mice","volume":"84","author":"Farr","year":"2003","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0050","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1002\/jnr.22006","article-title":"Combined hematopoietic and lentiviral gene-transfer therapies in newborn Twitcher mice reveal contemporaneous neurodegeneration and demyelination in Krabbe disease","volume":"87","author":"Galbiati","year":"2009","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0055","doi-asserted-by":"crossref","first-page":"1820","DOI":"10.1111\/j.1471-4159.2008.05279.x","article-title":"The role of AMPK in psychosine mediated effects on oligodendrocytes and astrocytes: implication for Krabbe disease","volume":"105","author":"Giri","year":"2008","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0065","doi-asserted-by":"crossref","first-page":"1428","DOI":"10.1046\/j.1471-4159.2003.01941.x","article-title":"Molecular mechanism of psychosine-induced cell death in human oligodendrocyte cell line","volume":"86","author":"Haq","year":"2003","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0060","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.bbrc.2006.02.131","article-title":"Dysfunction of peroxisomes in twitcher mice brain: a possible mechanism of psychosine-induced disease","volume":"343","author":"Haq","year":"2006","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0070","doi-asserted-by":"crossref","first-page":"R54","DOI":"10.1093\/hmg\/ddr112","article-title":"Combination therapies for lysosomal storage disease: is the whole greater than the sum of its parts?","volume":"20","author":"Hawkins-Salsbury","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0075","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1126\/science.6719111","article-title":"Progressive accumulation of toxic metabolite in a genetic leukodystrophy","volume":"224","author":"Igisu","year":"1984","journal-title":"Science"},{"key":"10.1016\/j.expneurol.2012.07.013_bb0080","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1002\/jnr.20529","article-title":"Peroxisomal participation in psychosine-mediated toxicity: implications for Krabbe's disease","volume":"80","author":"Khan","year":"2005","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0085","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1016\/0006-8993(80)90159-6","article-title":"The Twitcher mouse: an enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease)","volume":"202","author":"Kobayashi","year":"1980","journal-title":"Brain Res."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0090","doi-asserted-by":"crossref","first-page":"3610","DOI":"10.1523\/JNEUROSCI.6344-10.2011","article-title":"Macrophages counteract demyelination in a mouse model of globoid cell leukodystrophy","volume":"31","author":"Kondo","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0095","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1038\/sj.mt.6300026","article-title":"Central nervous system-directed AAV2\/5-mediated gene therapy synergizes with bone marrow transplantation in the murine model of globoid-cell leukodystrophy","volume":"15","author":"Lin","year":"2007","journal-title":"Mol. Ther."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0100","doi-asserted-by":"crossref","first-page":"15575","DOI":"10.1523\/JNEUROSCI.3579-11.2011","article-title":"The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis","volume":"31","author":"Macauley","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0105","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1002\/ana.23545","article-title":"Synergistic effects of central nervous system-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis","volume":"71","author":"Macauley","year":"2012","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0110","doi-asserted-by":"crossref","first-page":"2099","DOI":"10.1093\/carcin\/16.9.2099","article-title":"Uptake and distribution of N-acetylcysteine in mice: tissue-specific effects on glutathione concentrations","volume":"16","author":"McLellan","year":"1995","journal-title":"Carcinogenesis"},{"key":"10.1016\/j.expneurol.2012.07.013_bb0115","doi-asserted-by":"crossref","first-page":"1203","DOI":"10.1016\/S0891-5849(96)00554-0","article-title":"Blood glutathione as an index of radiation-induced oxidative stress in mice and humans","volume":"22","author":"Navarro","year":"1997","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0120","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/S0197-0186(99)00126-6","article-title":"In-vivo glutathione elevation protects against hydroxyl free radical-induced protein oxidation in rat brain","volume":"36","author":"Pocernich","year":"2000","journal-title":"Neurochem. Int."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0125","doi-asserted-by":"crossref","first-page":"9945","DOI":"10.1523\/JNEUROSCI.1802-11.2011","article-title":"Bone marrow transplantation augments the effect of brain- and spinal cord-directed adeno-associated virus 2\/5 gene therapy by altering inflammation in the murine model of globoid-cell leukodystrophy","volume":"31","author":"Reddy","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0135","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1002\/stem.555","article-title":"Mesenchymal lineage stem cells have pronounced anti-inflammatory effects in the twitcher mouse model of Krabbe's disease","volume":"29","author":"Ripoll","year":"2011","journal-title":"Stem Cells"},{"key":"10.1016\/j.expneurol.2012.07.013_bb0140","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1046\/j.1471-4159.1996.66031118.x","article-title":"Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease","volume":"66","author":"Sakai","year":"1996","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0145","first-page":"328","article-title":"Percutaneous intravenous injection in neonatal mice","volume":"49","author":"Sands","year":"1999","journal-title":"Lab. Anim. Sci."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0150","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1023\/A:1022436928925","article-title":"Twenty five years of the \u201cpsychosine hypothesis\u201d: a personal perspective of its history and present status","volume":"23","author":"Suzuki","year":"1998","journal-title":"Neurochem. Res."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0155","first-page":"255","article-title":"Physiological activity of psychosine","volume":"34","author":"Taketomi","year":"1964","journal-title":"Jpn. J. Exp. Med."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0160","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1016\/0003-2697(69)90064-5","article-title":"Enzymic method for quantitative determination of nanogram amounts of total and oxidized glutathione: applications to mammalian blood and other tissues","volume":"27","author":"Tietze","year":"1969","journal-title":"Anal. Biochem."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0165","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1038\/nm.2188","article-title":"Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy","volume":"16","author":"Viscomi","year":"2010","journal-title":"Nat. Med."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0170","doi-asserted-by":"crossref","first-page":"149","DOI":"10.2174\/1567202053586785","article-title":"Plasma infusions into porcine cerebral white matter induce early edema, oxidative stress, pro-inflammatory cytokine gene expression and DNA fragmentation: implications for white matter injury with increased blood\u2013brain-barrier permeability","volume":"2","author":"Wagner","year":"2005","journal-title":"Curr. Neurovasc. Res."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0175","series-title":"The Metabolic and Molecular Basis of Disease","first-page":"2669","article-title":"Galactosylceramide lipidosis: globoid cell leukodystrophy (Krabbe disease)","author":"Wenger","year":"2001"},{"key":"10.1016\/j.expneurol.2012.07.013_bb0180","doi-asserted-by":"crossref","first-page":"6068","DOI":"10.1523\/JNEUROSCI.5597-08.2009","article-title":"Psychosine accumulates in membrane microdomains in the brain of Krabbe patients, disrupting the raft architecture","volume":"29","author":"White","year":"2009","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2012.07.013_bb0185","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/j.neulet.2003.12.126","article-title":"Psychosine-induced apoptosis in a mouse oligodendrocyte progenitor cell line is mediated by caspase activation","volume":"358","author":"Zaka","year":"2004","journal-title":"Neurosci. Lett."}],"container-title":["Experimental Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488612002907?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488612002907?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T21:08:36Z","timestamp":1562015316000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0014488612002907"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,10]]},"references-count":36,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2012,10]]}},"alternative-id":["S0014488612002907"],"URL":"https:\/\/doi.org\/10.1016\/j.expneurol.2012.07.013","relation":{},"ISSN":["0014-4886"],"issn-type":[{"value":"0014-4886","type":"print"}],"subject":[],"published":{"date-parts":[[2012,10]]}}}