{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T22:06:17Z","timestamp":1790892377208,"version":"4.1.0"},"reference-count":57,"publisher":"Oxford University Press (OUP)","issue":"13","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2008,7,1]]},"DOI":"10.1093\/hmg\/ddn097","type":"journal-article","created":{"date-parts":[[2008,3,26]],"date-time":"2008-03-26T00:25:56Z","timestamp":1206491156000},"page":"1994-2005","source":"Crossref","is-referenced-by-count":57,"title":["Genetic background conversion ameliorates semi-lethality and permits behavioral analyses in cystathionine \u03b2-synthase-deficient mice, an animal model for hyperhomocysteinemia"],"prefix":"10.1093","volume":"17","author":[{"given":"Noriyuki","family":"Akahoshi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiho","family":"Kobayashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuki","family":"Ishizaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Izumi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiyuki","family":"Himi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makoto","family":"Suematsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isao","family":"Ishii","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2008,3,25]]},"reference":[{"key":"key\n\t\t\t\t20170522141437_DDN097C1","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1136\/heart.83.2.127","article-title":"Homocysteine, B vitamins, and risk of cardiovascular disease","volume":"83","author":"Robinson","year":"2000","journal-title":"Heart"},{"key":"key\n\t\t\t\t20170522141437_DDN097C2","first-page":"2007","article-title":"Disorders of transsulfuration","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Mudd","year":"2001","edition":"8th edn"},{"key":"key\n\t\t\t\t20170522141437_DDN097C3","first-page":"471","article-title":"Hyperhomocysteinemia: a new risk factor for degenerative diseases","volume":"48","author":"Herrmann","year":"2002","journal-title":"Clin. Lab."},{"key":"key\n\t\t\t\t20170522141437_DDN097C4","doi-asserted-by":"crossref","first-page":"1203","DOI":"10.1161\/hh1101.092180","article-title":"Endothelial dysfunction and elevation of S-adenosylhomocysteine in cystathionine \u03b2-synthase-deficient mice","volume":"88","author":"Dayal","year":"2001","journal-title":"Circ. Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C5","doi-asserted-by":"crossref","first-page":"S56","DOI":"10.1038\/sj.cdd.4401451","article-title":"Role of hyperhomocysteinemia in endothelial dysfunction and atherothrombotic disease","volume":"11","author":"Austin","year":"2004","journal-title":"Cell Death Differ."},{"key":"key\n\t\t\t\t20170522141437_DDN097C6","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1172\/JCI8342","article-title":"Endothelial dysfunction in a murine model of mild hyperhomocyst(e)inemia","volume":"106","author":"Eberhardt","year":"2000","journal-title":"J. Clin. Invest."},{"key":"key\n\t\t\t\t20170522141437_DDN097C7","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1136\/adc.38.201.425","article-title":"Homocystinuria: a new inborn error of metabolism associated with mental deficiency","volume":"38","author":"Carson","year":"1963","journal-title":"Arch. Dis. Child."},{"key":"key\n\t\t\t\t20170522141437_DDN097C8","doi-asserted-by":"crossref","first-page":"2994","DOI":"10.1016\/j.febslet.2006.04.088","article-title":"Mechanisms of homocysteine neurotoxicity in neurodegenerative diseases with special reference to dementia","volume":"580","author":"Obeid","year":"2006","journal-title":"FEBS Lett."},{"key":"key\n\t\t\t\t20170522141437_DDN097C9","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1002\/jnr.20514","article-title":"Homocysteic acid induces intraneuronal accumulation of neurotoxic A\u03b242: implications for the pathogenesis of Alzheimer\u2019s disease","volume":"80","author":"Hasegawa","year":"2005","journal-title":"J. Neurosci. Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C10","doi-asserted-by":"crossref","first-page":"35785","DOI":"10.1074\/jbc.M602799200","article-title":"A functional transsulfuration pathway in the brain links to glutathione homeostasis","volume":"281","author":"Vitvitsky","year":"2006","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C11","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1073\/pnas.92.5.1585","article-title":"Mice deficient in cystathionine \u03b2-synthase: animal models for mild and severe homocyst(e)inemia","volume":"92","author":"Watanabe","year":"1995","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522141437_DDN097C12","doi-asserted-by":"crossref","first-page":"52961","DOI":"10.1074\/jbc.M406820200","article-title":"Abnormal lipid metabolism in cystathionine \u03b2-synthase-deficient mice, an animal model for hyperhomocysteinemia","volume":"279","author":"Namekata","year":"2004","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C13","doi-asserted-by":"crossref","first-page":"1405","DOI":"10.1053\/j.gastro.2005.02.034","article-title":"Cystathionine beta synthase deficiency promotes oxidative stress, fibrosis, and steatosis in mice liver","volume":"128","author":"Robert","year":"2005","journal-title":"Gastroenterology"},{"key":"key\n\t\t\t\t20170522141437_DDN097C14","doi-asserted-by":"crossref","first-page":"12503","DOI":"10.1073\/pnas.231428998","article-title":"Overexpression of cellular glutathione peroxidase rescues homocyst(e)ine-induced endothelial dysfunction","volume":"98","author":"Weiss","year":"2001","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522141437_DDN097C15","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1152\/physiolgenomics.00199.2004","article-title":"Homocysteine levels in A\/J and C57BL\/6J mice: genetic, diet, gender, and parental effects","volume":"21","author":"Ernest","year":"2005","journal-title":"Physiol. Genomics"},{"key":"key\n\t\t\t\t20170522141437_DDN097C16","doi-asserted-by":"crossref","first-page":"1854","DOI":"10.1096\/fj.05-3724fje","article-title":"Cystathionine \u03b2-synthase, a key enzyme for homocysteine metabolism, is preferentially expressed in the radial glia\/astrocyte lineage of developing mouse CNS","volume":"19","author":"Enokido","year":"2005","journal-title":"FASEB J."},{"key":"key\n\t\t\t\t20170522141437_DDN097C17","doi-asserted-by":"crossref","first-page":"15227","DOI":"10.1073\/pnas.93.26.15227","article-title":"Homocysteine induces congenital defects of the heart and neural tube: effect of folic acid","volume":"93","author":"Rosenquist","year":"1996","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522141437_DDN097C18","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1146\/annurev.nutr.24.012003.132418","article-title":"Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine","volume":"24","author":"Stipanuk","year":"2004","journal-title":"Annu. Rev. Nutr."},{"key":"key\n\t\t\t\t20170522141437_DDN097C19","doi-asserted-by":"crossref","first-page":"1072","DOI":"10.1002\/ar.a.20082","article-title":"Hyperkeratosis in cystathionine beta synthase-deficient mice: an animal model of hyperhomocysteinemia","volume":"280","author":"Robert","year":"2004","journal-title":"Anat. Rec. A Discov. Mol. Cell. Evol. Biol."},{"key":"key\n\t\t\t\t20170522141437_DDN097C20","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1161\/hq1201.100456","article-title":"Cellular redox state and endothelial dysfunction in mildly hyperhomocysteinemic cystathionine \u03b2-synthase-deficient mice","volume":"22","author":"Weiss","year":"2002","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"key\n\t\t\t\t20170522141437_DDN097C21","doi-asserted-by":"crossref","first-page":"R39","DOI":"10.1152\/ajpregu.00036.2004","article-title":"Perturbations in homocysteine-linked redox homeostasis in a murine model for hyperhomocysteinemia","volume":"287","author":"Vitvitsky","year":"2004","journal-title":"Am. J. Physiol. Regul. Integr. Comp. Physiol."},{"key":"key\n\t\t\t\t20170522141437_DDN097C22","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1016\/j.pnpbp.2005.06.025","article-title":"The central nervous system in animal models of hyperhomocysteinemia","volume":"29","author":"Troen","year":"2005","journal-title":"Prog. Neuropsychopharmacol. Biol. Psychiatry"},{"key":"key\n\t\t\t\t20170522141437_DDN097C23","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1212\/WNL.58.10.1539","article-title":"Relationship between plasma homocysteine levels and brain atrophy in healthy elderly individuals","volume":"58","author":"Sachdev","year":"2002","journal-title":"Neurology"},{"key":"key\n\t\t\t\t20170522141437_DDN097C24","first-page":"21","article-title":"Preventive health care, 2000 update: screening and management of hyperhomocysteinemia for the prevention of coronary artery disease events. The Canadian Task Force on Preventive Health Care","volume":"163","author":"Booth","year":"2000","journal-title":"CMAJ"},{"key":"key\n\t\t\t\t20170522141437_DDN097C25","first-page":"682","article-title":"Amino acid metabolism","volume-title":"Fundamentals of Biochemistry; Life at the Molecular Level","author":"Voet","year":"2006","edition":"2nd edn"},{"key":"key\n\t\t\t\t20170522141437_DDN097C26","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1093\/ajcn\/46.4.593","article-title":"Newer concepts of the indispensable amino acids","volume":"46","author":"Laidlaw","year":"1987","journal-title":"Am. J. Clin. Nutr."},{"key":"key\n\t\t\t\t20170522141437_DDN097C27","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1042\/bj2440279","article-title":"Metabolism of cysteine in rat hepatocytes. Evidence for cysteinesulphinate-independent pathways","volume":"244","author":"Drake","year":"1987","journal-title":"Biochem. J."},{"key":"key\n\t\t\t\t20170522141437_DDN097C28","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1016\/S0022-2275(20)37196-0","article-title":"Cloning, expression, and chromosomal localization of mouse liver bile acid CoA:amino acid N-acyltransferase","volume":"38","author":"Falany","year":"1997","journal-title":"J. Lipid Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C29","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1016\/S0022-2275(20)38617-X","article-title":"Taurine increases bile acid pool size and reduces bile saturation index in the hamster","volume":"28","author":"Bellentani","year":"1987","journal-title":"J. Lipid Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C30","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1016\/j.lfs.2004.11.036","article-title":"Comparative regulation of major enzymes in the bile acid biosynthesis pathway by cholesterol, cholate and taurine in mice and rats","volume":"77","author":"Chen","year":"2005","journal-title":"Life Sci."},{"key":"key\n\t\t\t\t20170522141437_DDN097C31","doi-asserted-by":"crossref","first-page":"7636","DOI":"10.1074\/jbc.M309439200","article-title":"Hypertriglyceridemia in lecithin-cholesterol acyltransferase-deficient mice is associated with hepatic overproduction of triglycerides, increased lipogenesis, and improved glucose tolerance","volume":"279","author":"Ng","year":"2004","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C32","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1002\/bdrb.20071","article-title":"Failure of homocysteine to induce neural tube defects in a mouse model","volume":"77","author":"Bennett","year":"2006","journal-title":"Birth Defects Res. B Dev. Reprod. Toxicol."},{"key":"key\n\t\t\t\t20170522141437_DDN097C33","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1093\/hmg\/10.5.433","article-title":"Mice deficient in methylenetetrahydrofolate reductase exhibit hyperhomocysteinemia and decreased methylation capacity, with neuropathology and aortic lipid deposition","volume":"10","author":"Chen","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522141437_DDN097C34","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1007\/s00702-004-0154-7","article-title":"Studies on homocysteine plasma levels in Alzheimer\u2019s patients. Relevance for neurodegeneration","volume":"112","author":"Agnati","year":"2005","journal-title":"J. Neural. Transm."},{"key":"key\n\t\t\t\t20170522141437_DDN097C35","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1097\/00001756-200507130-00005","article-title":"Folate deficiency inhibits proliferation of adult hippocampal progenitors","volume":"16","author":"Kruman","year":"2005","journal-title":"Neuroreport"},{"key":"key\n\t\t\t\t20170522141437_DDN097C36","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1111\/j.1600-079X.2004.00144.x","article-title":"Melatonin alleviates behavioral deficits associated with apoptosis and cholinergic system dysfunction in the APP 695 transgenic mouse model of Alzheimer\u2019s disease","volume":"37","author":"Feng","year":"2004","journal-title":"J. Pineal. Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C37","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1016\/j.neurobiolaging.2006.05.035","article-title":"Impaired spatial memory in APP-overexpressing mice on a homocysteinemia-inducing diet","volume":"28","author":"Bernardo","year":"2007","journal-title":"Neurobiol. Aging"},{"key":"key\n\t\t\t\t20170522141437_DDN097C38","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/j.brainres.2005.04.011","article-title":"Melatonin improves learning and memory performances impaired by hyperhomocysteinemia in rats","volume":"1046","author":"Baydas","year":"2005","journal-title":"Brain Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C39","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.bbr.2005.06.047","article-title":"Different rankings of inbred mouse strains on the Morris maze and a refined 4-arm water escape task","volume":"165","author":"Wahlsten","year":"2005","journal-title":"Behav. Brain Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C40","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1016\/j.mehy.2007.02.034","article-title":"Prolonged stress will induce Alzheimer\u2019s disease in elderly people by increased release of homocysteic acid","volume":"69","author":"Hasegawa","year":"2007","journal-title":"Med. Hypotheses"},{"key":"key\n\t\t\t\t20170522141437_DDN097C41","doi-asserted-by":"crossref","first-page":"1678","DOI":"10.1515\/CCLM.2007.336","article-title":"Review: plasma protein homocysteinylation in uremia","volume":"45","author":"Perna","year":"2007","journal-title":"Clin. Chem. Lab. Med."},{"key":"key\n\t\t\t\t20170522141437_DDN097C42","doi-asserted-by":"crossref","first-page":"1704","DOI":"10.1161\/01.ATV.20.7.1704","article-title":"Homocysteine and its disulfide derivatives: a suggested consensus terminology","volume":"20","author":"Mudd","year":"2000","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"key\n\t\t\t\t20170522141437_DDN097C43","doi-asserted-by":"crossref","first-page":"3957","DOI":"10.1074\/jbc.275.6.3957","article-title":"Calcium-dependent human serum homocysteine thiolactone hydrolase. A protective mechanism against protein N-homocysteinylation","volume":"275","author":"Jakubowski","year":"2000","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C44","first-page":"392","article-title":"Protein homocysteinylation: a new mechanism of atherogenesis?","volume":"59","author":"Beltowski","year":"2005","journal-title":"Postepy High Med. Dosw. (Online)"},{"key":"key\n\t\t\t\t20170522141437_DDN097C45","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1373\/clinchem.2004.042531","article-title":"Urinary excretion of homocysteine-thiolactone in humans","volume":"51","author":"Chwatko","year":"2005","journal-title":"Clin. Chem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C46","doi-asserted-by":"crossref","first-page":"1707","DOI":"10.1096\/fj.06-7435com","article-title":"Mutations in methylenetetrahydrofolate reductase or cystathionine \u03b2-synthase gene, or a high-methionine diet, increase homocysteine thiolactone levels in humans and mice","volume":"21","author":"Chwatko","year":"2007","journal-title":"FASEB J."},{"key":"key\n\t\t\t\t20170522141437_DDN097C47","doi-asserted-by":"crossref","first-page":"4558","DOI":"10.1210\/jc.2004-0419","article-title":"Modification induced by homocysteine and low-density lipoprotein on human aortic endothelial cells: an in vitro study","volume":"89","author":"Vignini","year":"2004","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"key\n\t\t\t\t20170522141437_DDN097C48","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1002\/jcb.20069","article-title":"Effect of homocysteinylation of low density lipoproteins on lipid peroxidation of human endothelial cells","volume":"92","author":"Ferretti","year":"2004","journal-title":"J. Cell Biochem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C49","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1111\/j.1464-5491.2006.01809.x","article-title":"Homocysteinylation of low-density lipoproteins (LDL) from subjects with Type 1 diabetes: effect on oxidative damage of human endothelial cells","volume":"23","author":"Ferretti","year":"2006","journal-title":"Diabet. Med."},{"key":"key\n\t\t\t\t20170522141437_DDN097C50","doi-asserted-by":"crossref","first-page":"31504","DOI":"10.1074\/jbc.M213036200","article-title":"Altered gene expression in liver from a murine model of hyperhomocysteinemia","volume":"278","author":"Robert","year":"2003","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C51","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1006\/abio.1998.2949","article-title":"Separation of selenium-containing proteins in human and mouse plasma using tandem high-performance liquid chromatography columns coupled with inductively coupled plasma-mass spectrometry","volume":"267","author":"Koyama","year":"1999","journal-title":"Anal. Biochem."},{"key":"key\n\t\t\t\t20170522141437_DDN097C52","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1016\/S0022-2275(20)30123-1","article-title":"A new on-line dual enzymatic method for simultaneous quantification of cholesterol and triglycerides in lipoproteins by HPLC","volume":"43","author":"Usui","year":"2002","journal-title":"J. Lipid Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C53","doi-asserted-by":"crossref","first-page":"1799","DOI":"10.1248\/bpb.29.1799","article-title":"Differential induction of cystathionine \u03b3-lyase in the livers and kidneys of mouse dams during gestation and lactation","volume":"29","author":"Akahoshi","year":"2006","journal-title":"Biol. Pharm. Bull."},{"key":"key\n\t\t\t\t20170522141437_DDN097C54","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1042\/BJ20040243","article-title":"Murine cystathionine \u03b3-lyase: complete cDNA and genomic sequences, promoter activity, tissue distribution and developmental expression","volume":"381","author":"Ishii","year":"2004","journal-title":"Biochem. J."},{"key":"key\n\t\t\t\t20170522141437_DDN097C55","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/j.bbr.2006.01.018","article-title":"Comparisons between anxiety tests for selection of anxious and non anxious mice","volume":"169","author":"do-Rego","year":"2006","journal-title":"Behav. Brain Res."},{"key":"key\n\t\t\t\t20170522141437_DDN097C56","doi-asserted-by":"crossref","first-page":"1093S","DOI":"10.1093\/jn\/131.3.1093S","article-title":"Ameliorative effect of S-allylcysteine, a major thioallyl constituent in aged garlic extract, on learning deficits in senescence-accelerated mice","volume":"131","author":"Nishiyama","year":"2001","journal-title":"J. Nutr."},{"key":"key\n\t\t\t\t20170522141437_DDN097C57","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1046\/j.1460-9568.1998.00073.x","article-title":"A caspase inhibitor blocks ischaemia-induced delayed neuronal death in the gerbil","volume":"10","author":"Himi","year":"1998","journal-title":"Eur. J. Neurosci."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/17\/13\/1994\/17244790\/ddn097.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,6]],"date-time":"2021-09-06T13:31:56Z","timestamp":1630935116000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddn097"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,3,25]]},"references-count":57,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2008,3,25]]},"published-print":{"date-parts":[[2008,7,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddn097","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2008,3,25]]}}}