{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T23:40:37Z","timestamp":1793144437367,"version":"4.5.0"},"reference-count":70,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2009,9,23]],"date-time":"2009-09-23T00:00:00Z","timestamp":1253664000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cell Mol Neurobiol"],"published-print":{"date-parts":[[2010,3]]},"DOI":"10.1007\/s10571-009-9455-6","type":"journal-article","created":{"date-parts":[[2009,9,22]],"date-time":"2009-09-22T05:00:15Z","timestamp":1253595615000},"page":"317-326","source":"Crossref","is-referenced-by-count":67,"title":["Experimental Evidence that Phenylalanine Provokes Oxidative Stress in Hippocampus and Cerebral Cortex of Developing Rats"],"prefix":"10.1007","volume":"30","author":[{"given":"Carolina G.","family":"Fernandes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guilhian","family":"Leipnitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bianca","family":"Seminotti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre U.","family":"Amaral","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c2ngela","family":"Zanatta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen R.","family":"Vargas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos S.","family":"Dutra Filho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moacir","family":"Wajner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2009,9,23]]},"reference":[{"key":"9455_CR1","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/S0304-3940(01)01636-6","volume":"302","author":"MY Aksenov","year":"2001","unstructured":"Aksenov MY, Markesbery WR (2001) Changes in thiol content and expression of glutathione redox system genes in the hippocampus and cerebellum in Alzheimer\u2019s disease. Neurosci Lett 302:141\u2013145","journal-title":"Neurosci Lett"},{"key":"9455_CR2","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1007\/BF00692698","volume":"58","author":"ML Bauman","year":"1982","unstructured":"Bauman ML, Kemper TL (1982) Morphologic and histoanatomic observations of the brain in untreated human phenylketonuria. Acta Neuropathol 58:55\u201363","journal-title":"Acta Neuropathol"},{"key":"9455_CR3","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/S0891-5849(02)00883-3","volume":"33","author":"C Behl","year":"2002","unstructured":"Behl C, Moosmann B (2002a) Antioxidant neuroprotection in Alzheimer\u2019s disease as preventive and therapeutic approach. Free Radic Biol Med 33:182\u2013191","journal-title":"Free Radic Biol Med"},{"key":"9455_CR4","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1515\/BC.2002.053","volume":"383","author":"C Behl","year":"2002","unstructured":"Behl C, Moosmann B (2002b) Oxidative nerve cell death in Alzheimer\u2019s disease and stroke: antioxidants as neuroprotective compounds. Biol Chem 383:521\u2013536","journal-title":"Biol Chem"},{"key":"9455_CR5","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1097\/01.rmr.0000245461.90406.ad","volume":"17","author":"D Berg","year":"2006","unstructured":"Berg D, Youdim MB (2006) Role of iron in neurodegenerative disorders. Top Magn Reson Imaging 17:5\u201317","journal-title":"Top Magn Reson Imaging"},{"key":"9455_CR6","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1016\/S0140-6736(53)90473-5","volume":"265","author":"H Bickel","year":"1953","unstructured":"Bickel H, Gerrard J, Hickmans EM (1953) Influence of phenylalanine intake on phenylketonuria. Lancet 265:812\u2013813","journal-title":"Lancet"},{"key":"9455_CR7","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1007\/BF00711745","volume":"18","author":"S Bird","year":"1995","unstructured":"Bird S, Miller NJ, Collins JE, Rice-Evans CA (1995) Plasma antioxidant capacity in two cases of tyrosinemia type 1: one case treated with NTBC. J Inherit Metab Dis 18:123\u2013126","journal-title":"J Inherit Metab Dis"},{"key":"9455_CR8","doi-asserted-by":"crossref","first-page":"1246","DOI":"10.1046\/j.1471-4159.2001.00689.x","volume":"79","author":"MB Bogdanov","year":"2001","unstructured":"Bogdanov MB, Andreassen OA, Dedeoglu A, Ferrante RJ, Beal MF (2001) Increased oxidative damage to DNA in a transgenic mouse model of Huntington\u2019s disease. J Neurochem 79:1246\u20131249","journal-title":"J Neurochem"},{"key":"9455_CR9","first-page":"347","volume":"108","author":"RW Browne","year":"1998","unstructured":"Browne RW, Armstrong D (1998) Reduced glutathione and glutathione disulfide. Methods Mol Biol 108:347\u2013352","journal-title":"Methods Mol Biol"},{"key":"9455_CR10","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/S0025-7753(00)71479-5","volume":"115","author":"C Colome","year":"2000","unstructured":"Colome C, Sierra C, Vilaseca MA (2000) Congenital errors of metabolism: cause of oxidative stress? Med Clin 115:11\u2013117","journal-title":"Med Clin"},{"key":"9455_CR11","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/S0736-5748(02)00108-9","volume":"21","author":"E Costabeber","year":"2003","unstructured":"Costabeber E, Kessler A, Dutra-Filho CS, Wyse ATS, Wajner M, Wannmacher CMD (2003) Hyperfenilalaninemia reduces creatine kinase activity in the cerebral cortex of rats. Int J Dev Neurosci 21:111\u2013116","journal-title":"Int J Dev Neurosci"},{"key":"9455_CR12","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1007\/BF01800019","volume":"15","author":"G Darling","year":"1992","unstructured":"Darling G, Mathias P, O\u2019Regan M, Naughten E (1992) Serum selenium levels in individuals on PKU diets. J Inherit Metab Dis 15:769\u2013773","journal-title":"J Inherit Metab Dis"},{"key":"9455_CR13","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/S0006-8993(02)04118-5","volume":"964","author":"F Oliveira Marques de","year":"2003","unstructured":"de Oliveira Marques F, Hagen ME, Pederzolli CD (2003) Glutaric acid induces oxidative stress in brain of young rats. Brain Res 964:153\u2013158","journal-title":"Brain Res"},{"key":"9455_CR14","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1016\/j.brainres.2006.09.101","volume":"1127","author":"JE Embury","year":"2007","unstructured":"Embury JE, Charron CE, Martynyuk A, Zori AG, Liu B, Ali SF, Rowland NE, Laipis PJ (2007) PKU is a reversible neurodegenerative process within the nigrostriatum that begins as early as 4\u00a0weeks of age in Path(enu2) mice. Brain Res 1127:136\u2013150","journal-title":"Brain Res"},{"key":"9455_CR15","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1016\/S0891-5849(02)00781-5","volume":"32","author":"N Ercal","year":"2002","unstructured":"Ercal N, Aykin-Burns N, Gurer-Orhan H, McDonald JD (2002) Oxidative stress in a phenylketonuria animal model. Free Radic Biol Med 32:906\u2013911","journal-title":"Free Radic Biol Med"},{"key":"9455_CR16","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/0076-6879(90)86134-H","volume":"186","author":"H Esterbauer","year":"1990","unstructured":"Esterbauer H, Cheeseman KH (1990) Determination of aldehydic lipid peroxidation products: malonaldehyde and 4-hydroxynonenal. Methods Enzymol 186:407\u2013421","journal-title":"Methods Enzymol"},{"key":"9455_CR17","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1006\/abbi.2001.2292","volume":"388","author":"P Evelson","year":"2001","unstructured":"Evelson P, Travacio M, Repetto M, Escobar J, Llesuy S, Lissi EA (2001) Evaluation of total reactive antioxidant potential (TRAP) of tissue homogenates and their cytosols. Arch Biochem Biophys 388:261\u2013266","journal-title":"Arch Biochem Biophys"},{"key":"9455_CR18","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/S1357-2725(02)00275-3","volume":"35","author":"MR Fighera","year":"2003","unstructured":"Fighera MR, Bonini JS, de Oliveira TG, Frussa-Filho R, Dutra-Filho CS, Rubin MA, Mello CF (2003) GM1 ganglioside attenuates convulsions and thiobarbituric acid reactive substances production induced by the intraestriatal injection of methylmalonic acid. Int J Biochem Cell Biol 35:465\u2013473","journal-title":"Int J Biochem Cell Biol"},{"key":"9455_CR19","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1515\/bchm2.1934.227.1-4.169","volume":"227","author":"A F\u00f8lling","year":"1934","unstructured":"F\u00f8lling A (1934) \u00dcber Ausscheidung von Phenylbrenztraubens\u00e4ure in den Harn als Stoffwechseanomalie in Verbindung mit Imbezillit\u00e4t. Z Physiol Chem 227:169\u2013176","journal-title":"Z Physiol Chem"},{"key":"9455_CR20","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1097\/00001756-200002280-00023","volume":"11","author":"FU Fontella","year":"2000","unstructured":"Fontella FU, Pulrolnik V, Gassen E, Wannmacher CMD, Klein AB, Wajner M, Dutra-Filho CS (2000) Propionic and l-methylmalonic acids induce oxidative stress in brain of young rats. Neuroreport 11:541\u2013544","journal-title":"Neuroreport"},{"key":"9455_CR21","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1002\/jnr.10569","volume":"72","author":"AV Glushakov","year":"2003","unstructured":"Glushakov AV, Dennis DM, Summers C, Seubert CN, Martynyuk AE (2003) l-Phenylalanine selectively depress currents at glutamatergic excitatory synapses. J Neurosci Res 72:116\u2013124","journal-title":"J Neurosci Res"},{"key":"9455_CR22","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1093\/brain\/awh354","volume":"128","author":"AV Glushakov","year":"2005","unstructured":"Glushakov AV, Glushakova O, Varshney M, Bajpai LK, Summers C, Laipis PJ, Embury JE, Baker SP, Otero DH, Dennis DM, Seubert CN, Martynyuk AE (2005) Long-term changes in glutamatergic synaptic transmission in phenylketonuria. Brain 128:300\u2013307","journal-title":"Brain"},{"key":"9455_CR23","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1016\/S0925-4439(01)00112-0","volume":"1586","author":"MEK Hagen","year":"2002","unstructured":"Hagen MEK, Pederzolli CD, Sgaravatti AM, Bridi R, Wajner M, Wannmacher CMD, Wyse ATS, Dutra-Filho CS (2002) Experimental hyperphenylalaninemia provokes oxidative stress in rat brain. Biochim Biophys Acta 1586:344\u2013352","journal-title":"Biochim Biophys Acta"},{"key":"9455_CR24","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/0166-2236(85)90010-4","volume":"8","author":"B Halliwell","year":"1996","unstructured":"Halliwell B, Gutteridge JMC (1996) Oxygen radicals and nervous system. Trends Neurosci 8:22\u201326","journal-title":"Trends Neurosci"},{"key":"9455_CR25","first-page":"268","volume-title":"Free radicals in biology and medicine","author":"B Halliwell","year":"2007","unstructured":"Halliwell B, Gutteridge JMC (2007) Measurement of reactive species. In: Halliwell B, Gutteridge JMC (eds) Free radicals in biology and medicine. Oxford University Press, Oxford, pp 268\u2013340"},{"key":"9455_CR26","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1203\/00006450-199607000-00004","volume":"40","author":"S Hasselbalch","year":"1996","unstructured":"Hasselbalch S, Knudsen GM, Toft PB, H\u00f8gh P, Tedeschi E, Holm S, Videbaek C, Henriksen O, Lou HC, Paulson OB (1996) Cerebral glucose metabolism is decreased in white matter changes in patients with phenylketonuria. Pediatr Res 40:21\u201324","journal-title":"Pediatr Res"},{"key":"9455_CR27","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1203\/01.pdr.0000203091.45988.8d","volume":"59","author":"F H\u00f6rster","year":"2006","unstructured":"H\u00f6rster F, Schwab MA, Sauer SW, Pietz J, Hoffmann GF, Okun JG, K\u00f6lker S, Kins S (2006) Phenylalanine reduces synaptic density in mixed cortical cultures from mice. Pediatr Res 59:544\u2013548","journal-title":"Pediatr Res"},{"key":"9455_CR28","doi-asserted-by":"crossref","first-page":"S102","DOI":"10.1007\/PL00014371","volume":"159","author":"PR Huttenlocher","year":"2000","unstructured":"Huttenlocher PR (2000) The neuropathology of phenylketonuria: human and animal studies. Eur J Pediatr 159:S102\u2013S106","journal-title":"Eur J Pediatr"},{"key":"9455_CR29","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1046\/j.1471-4159.2003.01887.x","volume":"86","author":"B Joseph","year":"2003","unstructured":"Joseph B, Dyer CA (2003) Relationship between myelin production and dopamine synthesis in the PKU mouse brain. J Neurochem 86:615\u2013626","journal-title":"J Neurochem"},{"key":"9455_CR30","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1023\/A:1010942929678","volume":"26","author":"E Karelson","year":"2001","unstructured":"Karelson E, Bogdanovic N, Garlind A, Winblad B, Zilmer K, Kullisaar T, Vihalemm T, Kairane C, Zilmer M (2001) The cerebrocortical areas in normal brain aging and Alzheimer\u2019s disease: noticeable differences in the lipid peroxidation level and in antioxidant defense. Neurochem Res 26:353\u2013361","journal-title":"Neurochem Res"},{"key":"9455_CR31","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.1046\/j.0953-816x.2001.01595.x","volume":"13","author":"S K\u00f6lker","year":"2001","unstructured":"K\u00f6lker S, Ahlemeyer B, Huhne R, Mayatepek E, Krieglstein J, Hoffmann GF (2001a) Potentiation of 3-hydroxyglutarate neurotoxicity following induction of astrocytic iNOS in neonatal rat hippocampal cultures. Eur J Neurosci 13:2115\u20132122","journal-title":"Eur J Neurosci"},{"key":"9455_CR32","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1203\/00006450-200107000-00015","volume":"50","author":"S K\u00f6lker","year":"2001","unstructured":"K\u00f6lker S, Ahlemeyer B, Krieglstein J, Hoffmann GF (2001b) Contribution of reactive oxygen species to 3-hydroxyglutarate neurotoxicity in primary neuronal cultures from chick embryo telencephalons. Pediatr Res 50:76\u201382","journal-title":"Pediatr Res"},{"key":"9455_CR33","doi-asserted-by":"crossref","first-page":"10289","DOI":"10.1523\/JNEUROSCI.19-23-10289.1999","volume":"19","author":"DM Kuhn","year":"1999","unstructured":"Kuhn DM, Aretha CW, Geddes TJ (1999) Peroxynitrite inactivation of tyrosine hydroxylase: mediation by sulfhydryl oxidation, not tyrosine nitration. J Neurosci 19:10289\u201310294","journal-title":"J Neurosci"},{"key":"9455_CR34","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1002\/jnr.10735","volume":"74","author":"A Latini","year":"2003","unstructured":"Latini A, Scussiato K, Rosa RB, Leipnitz G, Llesuy S, Bell\u00f3-Klein A, Dutra-Filho CS, Wajner M (2003a) Induction of oxidative stress by l-2-hydroxyglutaric acid in rat brain. J Neurosci Res 74:103\u2013110","journal-title":"J Neurosci Res"},{"key":"9455_CR35","doi-asserted-by":"crossref","first-page":"2017","DOI":"10.1046\/j.1460-9568.2003.02639.x","volume":"17","author":"A Latini","year":"2003","unstructured":"Latini A, Scussiato K, Rosa RB, Llesuy S, Bell\u00f3-Klein A, Dutra-Filho CS, Wajner M (2003b) d-2-Hydroxyglutaric acid induces oxidative stress in cerebral c\u00f3rtex of young rats. Eur J Neurosci 17:2017\u20132022","journal-title":"Eur J Neurosci"},{"key":"9455_CR36","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/S0021-9258(19)52451-6","volume":"193","author":"OH Lowry","year":"1951","unstructured":"Lowry OH, Rosebrough NJ, Lewis-Farr A, Randall RJ (1951) Protein measurement with the Folin phenol reagent. J Biol Chem 193:265\u2013275","journal-title":"J Biol Chem"},{"key":"9455_CR37","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.2741\/2130","volume":"12","author":"C Mancuso","year":"2007","unstructured":"Mancuso C, Scapagini G, Curr\u00f2 D, Giuffrida Stella AM, De Marco C, Butterfield DA, Calabrese V (2007) Mitochondrial dysfunction, free radical generation and cellular stress response in neurodegenerative disorders. Front Biosci 12:1107\u20131123","journal-title":"Front Biosci"},{"key":"9455_CR38","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1002\/jnr.10307","volume":"69","author":"F Martinez-Cruz","year":"2002","unstructured":"Martinez-Cruz F, Pozo D, Osuna C, Espinar A, Marchante C, Guerrero JM (2002) Oxidative stress induced by phenylketonuria in the rat: prevention by melatonin, vitamin E and vitamin C. J Neurosci Res 69:550\u2013558","journal-title":"J Neurosci Res"},{"key":"9455_CR39","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/s10545-007-0556-4","volume":"30","author":"R Matalon","year":"2007","unstructured":"Matalon R, Michals-Matalon K, Bhatia G, Burlina AB, Burlina AP, Braga C, Fiori L, Giovannini M, Grechanina E, Novikov P, Grady J, Tyring SK, Guttler F (2007) Double blind placebo control trial of large neutral amino acids in treatment of PKU: effect on blood phenylalanine. J Inherit Metab Dis 30:153\u2013158","journal-title":"J Inherit Metab Dis"},{"key":"9455_CR40","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1016\/S0891-5849(01)00679-7","volume":"31","author":"E M\u00e9ndez-Alvarez","year":"2001","unstructured":"M\u00e9ndez-Alvarez E, Soto-Otero R, Herminda-Ameijeiras A, L\u00f3pez-Mart\u00edn ME, Labandeira-Garc\u00eda JL (2001) Effect of iron and manganese on hydroxyl radical production by 6-hydroxydopamine: mediation of antioxidants. Free Radic Biol Med 31:986\u2013998","journal-title":"Free Radic Biol Med"},{"key":"9455_CR41","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1006\/niox.2000.0319","volume":"5","author":"KM Miranda","year":"2001","unstructured":"Miranda KM, Espey MG, Wink DA (2001) A rapid, simple spectrophotometric method for simultaneous detection of nitrate and nitrite. Nitric Oxide 5:62\u201371","journal-title":"Nitric Oxide"},{"key":"9455_CR42","doi-asserted-by":"crossref","first-page":"S121","DOI":"10.1007\/PL00014374","volume":"159","author":"HE M\u00f6ller","year":"2000","unstructured":"M\u00f6ller HE, Ullrich K, Weglage J (2000) In vivo proton magnetic resonance spectroscopy in phenylketonuria. Eur J Pediatr 159:S121\u2013S125","journal-title":"Eur J Pediatr"},{"key":"9455_CR43","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1542\/peds.112.S4.1580","volume":"112","author":"HE M\u00f6ller","year":"2003","unstructured":"M\u00f6ller HE, Weglage J, Bick U, Wiedermann D, Feldmann R, Ullrich K (2003) Brain imaging and proton magnetic resonance spectroscopy in patients with phenylketonuria. Pediatrics 112:1580\u20131583","journal-title":"Pediatrics"},{"key":"9455_CR44","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/S0009-8981(97)00061-2","volume":"263","author":"D Moyano","year":"1997","unstructured":"Moyano D, Vilaseca MA, Pineda M, Campistol J, Vernet A, P\u00f3o P, Artuch R, Sierra C (1997) Tocopherol in inborn errors of intermediary metabolism. Clin Chim Acta 263:147\u2013155","journal-title":"Clin Chim Acta"},{"key":"9455_CR45","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1023\/A:1022482604276","volume":"23","author":"WM Pardridge","year":"1998","unstructured":"Pardridge WM (1998) Blood\u2013brain barrier carrier-mediated transport and brain metabolism of amino acids. Neurochem Res 23:635\u2013644","journal-title":"Neurochem Res"},{"key":"9455_CR46","doi-asserted-by":"crossref","first-page":"234","DOI":"10.1016\/S0006-8993(00)02082-5","volume":"862","author":"F P\u00e9rez-Severiano","year":"2000","unstructured":"P\u00e9rez-Severiano F, Rios C, Segovia J (2000) Striatal oxidative damage parallels the expression of a neurological phenotype in mice transgenic for the mutation of Huntington\u2019s disease. Brain Res 862:234\u2013237","journal-title":"Brain Res"},{"key":"9455_CR47","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1203\/01.PDR.0000055867.83310.9E","volume":"53","author":"J Pietz","year":"2003","unstructured":"Pietz J, Rupp A, Ebinger F, Rating D, Mayatepek E, Boesch C, Kreis R (2003) Cerebral energy metabolism in phenylketonuria: findings by quantitative in vivo 31P MR spectroscopy. Pediatr Res 53:654\u2013662","journal-title":"Pediatr Res"},{"key":"9455_CR48","first-page":"203","volume":"24","author":"OEA Pratt","year":"1980","unstructured":"Pratt OEA (1980) New approach to the treatment of phenylketonuria. J Ment Defic Res 24:203\u2013217","journal-title":"J Ment Defic Res"},{"key":"9455_CR49","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1023\/A:1015529511664","volume":"27","author":"VC Rech","year":"2002","unstructured":"Rech VC, Feksa LR, Dutra-Filho CS, Wyse ATS, Wajner M, Wannmacher CM (2002) Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex. Neurochem Res 27:353\u2013357","journal-title":"Neurochem Res"},{"key":"9455_CR50","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1006\/abio.2000.4542","volume":"280","author":"CN Sarkissian","year":"2000","unstructured":"Sarkissian CN, Scriver CR, Mamer OA (2000) Measurement of phenyllactate, phenylacetate, and phenylpyruvate by negative ion chemical ionization-gas chromatography\/mass spectrometry in brain of mouse genetic models of phenylketonuria and non-phenylketonuria hyperphenylalaninemia. Anal Biochem 280:242\u2013249","journal-title":"Anal Biochem"},{"key":"9455_CR51","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1016\/j.nbd.2008.07.020","volume":"32","author":"U Schumacher","year":"2008","unstructured":"Schumacher U, Lukacs Z, Kaltschmidt C, Freudlsperger C, Schulz D, Kompisch K, M\u00fcller R, Rudolph T, Santer R, Lorke DE, Ullrich K (2008) High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: implications for the pathophysiology of phenylketonuria. Neurobiol Dis 32:385\u2013390","journal-title":"Neurobiol Dis"},{"key":"9455_CR52","first-page":"1667","volume-title":"The metabolic and molecular bases of inherited disease","author":"CR Scriver","year":"2001","unstructured":"Scriver CR (2001) Hyperphenylalaninemia: phenylalanine hydroxylase deficiency. In: Scriver CR, Beaudet AL, Sly WS, Valle D, Childs B, Kinzler KW, Vogelstein B (eds) The metabolic and molecular bases of inherited disease, 8th edn. McGraw-Hill, New York, pp 1667\u20131724","edition":"8"},{"key":"9455_CR53","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1002\/1097-4547(20000901)61:5<549::AID-JNR10>3.0.CO;2-0","volume":"61","author":"S Shefer","year":"2000","unstructured":"Shefer S, Tint GS, Jean-Guillaume D, Daikhin E, Kendler A, Nguyen LB, Yudkoff M, Dyer CA (2000) Is there a relationship between 3-hydroxy-3-methylglutaryl coenzyme a reductase activity and forebrain pathology in PKU mouse? J Neurosci Res 61:549\u2013563","journal-title":"J Neurosci Res"},{"key":"9455_CR54","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0009-8981(98)00091-6","volume":"276","author":"C Sierra","year":"1998","unstructured":"Sierra C, Vilaseca MA, Moyano D, Brandi N, Campistol J, Lambruschini N, Cambra FJ, Deulofeu R, Mira A (1998) Antioxidant status in hyperphenylalaninemia. Clin Chim Acta 276:1\u20139","journal-title":"Clin Chim Acta"},{"key":"9455_CR55","doi-asserted-by":"crossref","first-page":"S236","DOI":"10.1016\/S0387-7604(01)00369-2","volume":"23","author":"C Sierra","year":"2001","unstructured":"Sierra C, Vilaseca MA, Brandi N, Artuch R, Mira A, Nieto M, Pineda M (2001) Oxidative stress in Rett syndrome. Brain Dev 23:S236\u2013S239","journal-title":"Brain Dev"},{"key":"9455_CR56","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.bbadis.2005.02.005","volume":"1740","author":"LR Sirtori","year":"2005","unstructured":"Sirtori LR, Dutra-Filho CS, Fitarelli D, Sitta A, Haeser A, Barschak AG, Wajner M, Coelho DM, Llesuy S, Bell\u00f3-Klein A, Giugliani R, Deon M, Vargas CR (2005) Oxidative stress in patients with phenylketonuria. Biochim Biophys Acta 1740:68\u201373","journal-title":"Biochim Biophys Acta"},{"key":"9455_CR57","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1007\/s11011-006-9035-0","volume":"21","author":"A Sitta","year":"2006","unstructured":"Sitta A, Barschak AG, Deon M, Terroso T, Pires R, Giugliani R, Dutra-Filho CS, Wajner M, Vargas CR (2006) Investigation of oxidative stress parameters in treated phenylketonuric patients. Metab Brain Dis 21:287\u2013296","journal-title":"Metab Brain Dis"},{"key":"9455_CR58","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.ijdevneu.2009.01.001","volume":"27","author":"A Sitta","year":"2009","unstructured":"Sitta A, Barschak AG, Deon M, Barden AT, Biancini GB, Vargas PR, de Souza CF, Netto C, Wajner M, Vargas CR (2009a) Effect of short- and long-term exposition to high phenylalanine blood levels on oxidative damage in phenylketonuric patients. Int J Dev Neurosci 27:243\u2013247","journal-title":"Int J Dev Neurosci"},{"key":"9455_CR59","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1007\/s10571-008-9313-y","volume":"29","author":"A Sitta","year":"2009","unstructured":"Sitta A, Barschak AG, Deon M, de Mari JF, Barden AT, Vanzin CS, Biancini GB, Schwartz IV, Wajner M, Vargas CR (2009b) l-Carnitine blood levels and oxidative stress in treated phenylketonuric patients. Cell Mol Neurobiol 29:211\u2013218","journal-title":"Cell Mol Neurobiol"},{"key":"9455_CR60","doi-asserted-by":"crossref","first-page":"1651","DOI":"10.1111\/j.1471-4159.1987.tb01039.x","volume":"49","author":"QR Smith","year":"1987","unstructured":"Smith QR, Momma S, Aoyagi M, Rapoport SI (1987) Kinetics of neutral amino acid transport across the blood\u2013brain barrier. J Neurochem 49:1651\u20131658","journal-title":"J Neurochem"},{"key":"9455_CR61","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1111\/j.1471-4159.2005.03070.x","volume":"93","author":"N Stoy","year":"2005","unstructured":"Stoy N, Mackay GM, Forrest CM, Christofides J, Egerton M, Stone TW, Darlington LG (2005) Tryptophan metabolism and oxidative stress in patients with Huntington\u2019s disease. J Neurochem 93:611\u2013623","journal-title":"J Neurochem"},{"key":"9455_CR62","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1023\/A:1013907104585","volume":"26","author":"EL Streck","year":"2001","unstructured":"Streck EL, Zugno AI, Tagliari B, Franzon R, Wannmacher CM, Wajner M, Wyse ATS (2001) Inhibition of rat brain Na+, K+-ATPase activity induced by homocysteine is probably mediated by oxidative stress. Neurochem Res 26:1195\u20131200","journal-title":"Neurochem Res"},{"key":"9455_CR63","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1023\/A:1023815119931","volume":"18","author":"EL Streck","year":"2003","unstructured":"Streck EL, Vieira PS, Wannmacher CMD, Dutra-Filho CS, Wajner M, Wyse ATS (2003) In vitro effect of homocysteine on some parameters of oxidative stress in rat hippocampus. Metab Brain Dis 18:147\u2013154","journal-title":"Metab Brain Dis"},{"key":"9455_CR64","doi-asserted-by":"crossref","first-page":"S109","DOI":"10.1007\/PL00014370","volume":"159","author":"R Surtees","year":"2000","unstructured":"Surtees R, Blau N (2000) The neurochemistry of phenylketonuria. Eur J Pediatr 159:S109\u2013S113","journal-title":"Eur J Pediatr"},{"key":"9455_CR65","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1093\/ajcn\/73.2.153","volume":"73","author":"FJ Spronsen van","year":"2001","unstructured":"van Spronsen FJ, van Rijn M, Bekhof J, Koch R, Smit PG (2001) Phenylketonuria: tyrosine supplementation in phenylalanine-restricted diets. Am J Clin Nutr 73:153\u2013157","journal-title":"Am J Clin Nutr"},{"key":"9455_CR66","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1007\/s10545-008-0946-2","volume":"32","author":"FJ Spronsen van","year":"2009","unstructured":"van Spronsen FJ, Hoeksma M, Reijngoud DJ (2009) Brain dysfunction in phenylketonuria: is phenylalanine toxicity the only possible case? J Inherit Metab Dis 32:46\u201351","journal-title":"J Inherit Metab Dis"},{"key":"9455_CR67","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.bbadis.2003.10.004","volume":"1688","author":"CR Vargas","year":"2004","unstructured":"Vargas CR, Wajner M, Sirtori LR, Goulart L, Chiochetta M, Coelho D, Latini A, Llesuy S, Bell\u00f3-Klein A, Giugliani R, Deon M, Mello CF (2004) Evidence that oxidative stress is increased in patients with X-linked adrenoleukodystrophy. Biochim Biophys Acta 1688:26\u201332","journal-title":"Biochim Biophys Acta"},{"key":"9455_CR68","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1023\/B:BOLI.0000037353.13085.e2","volume":"27","author":"M Wajner","year":"2004","unstructured":"Wajner M, Latini A, Wyse ATS, Dutra-Filho CS (2004) The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies. J Inherit Metab Dis 27:427\u2013448","journal-title":"J Inherit Metab Dis"},{"key":"9455_CR69","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/0009-8981(92)90157-L","volume":"207","author":"BC Wilke","year":"1992","unstructured":"Wilke BC, Vidailhet M, Favier A, Guillemin C, Ducros V, Arnaud J, Richard MJ (1992) Selenium, glutathione peroxidase (GSH-Px) and lipid peroxidation products before an after selenium supplementation. Clin Chim Acta 207:137\u2013142","journal-title":"Clin Chim Acta"},{"key":"9455_CR70","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1111\/j.1469-8749.1976.tb04212.x","volume":"18","author":"B Wood","year":"1976","unstructured":"Wood B (1976) Neurological disturbance in a phenylketonuric child after discontinuation of dietary treatment. Dev Med Child Neurol 18:657\u2013665","journal-title":"Dev Med Child Neurol"}],"container-title":["Cellular and Molecular Neurobiology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10571-009-9455-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10571-009-9455-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10571-009-9455-6","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,26]],"date-time":"2023-05-26T21:16:25Z","timestamp":1685135785000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s10571-009-9455-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,9,23]]},"references-count":70,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2010,3]]}},"alternative-id":["9455"],"URL":"https:\/\/doi.org\/10.1007\/s10571-009-9455-6","relation":{},"ISSN":["0272-4340","1573-6830"],"issn-type":[{"value":"0272-4340","type":"print"},{"value":"1573-6830","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,9,23]]}}}