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The patients present mild\u2010to\u2010moderate mental retardation with exaggerated affective responses, periodic mood swings, and apparent hypersexual behavior. Creatine kinase plays a key role in energy metabolism of tissues with intermittently high and fluctuating energy requirements, such as nervous tissue. The main objective of the present study was to investigate the effect of acute administration of tryptophan on creatine kinase activity in brain cortex of Wistar rats. We also studied the in vitro effect of this amino acid on creatine kinase activity in the brain cortex of non\u2010treated rats. The results indicated that tryptophan inhibits creatine kinase in vitro and in vivo. We also observed that the in vitro inhibition was fully prevented but not reversed by pre\u2010incubation with reduced glutathione, suggesting that the inhibitory effect of tryptophan on CK activity is possibly mediated by oxidation of essential thiol groups of the enzyme and\/or long\u2010lasting adduct formation. Considering the importance of creatine kinase for the maintenance of energy homeostasis in the brain, it is conceivable that an inhibition of this enzyme activity in the brain may be one of the mechanisms by which tryptophan might be neurotoxic.<\/jats:p><\/jats:sec>","DOI":"10.1016\/j.ijdevneu.2003.12.004","type":"journal-article","created":{"date-parts":[[2004,2,6]],"date-time":"2004-02-06T10:17:37Z","timestamp":1076062657000},"page":"95-101","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Tryptophan reduces creatine kinase activity in the brain cortex of rats"],"prefix":"10.1002","volume":"22","author":[{"given":"Andrea Renata","family":"Cornelio","sequence":"first","affiliation":[{"name":"Departamento de Bioqu\u0131\u0301mica Instituto de Ci\u00eancias B\u00e1sicas da Sa\u00fade Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos 2600 CEP 90.035\u2010003 Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr","given":"Valn\u00eas","family":"Rodrigues","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u0131\u0301mica Instituto de Ci\u00eancias B\u00e1sicas da Sa\u00fade Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos 2600 CEP 90.035\u2010003 Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela Terezinha","family":"de Souza Wyse","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u0131\u0301mica Instituto de Ci\u00eancias B\u00e1sicas da Sa\u00fade Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos 2600 CEP 90.035\u2010003 Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlos Severo","family":"Dutra\u2010Filho","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u0131\u0301mica Instituto de Ci\u00eancias B\u00e1sicas da Sa\u00fade Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos 2600 CEP 90.035\u2010003 Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Moacir","family":"Wajner","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u0131\u0301mica Instituto de Ci\u00eancias B\u00e1sicas da Sa\u00fade Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos 2600 CEP 90.035\u2010003 Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clovis Milton Duval","family":"Wannmacher","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u0131\u0301mica Instituto de Ci\u00eancias B\u00e1sicas da Sa\u00fade Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos 2600 CEP 90.035\u2010003 Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,2,5]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2000.0742520.x"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1006\/exnr.1997.6550"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1159\/000017098"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1006\/jmcc.1998.0662"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2001.00052.x"},{"key":"e_1_2_6_7_1","first-page":"54","article-title":"The action of acute alcoholic intoxication on antioxidant system and creatine kinase activity in the brain of rat embryos","volume":"55","author":"Burmistrov S.O.","year":"1992","journal-title":"Eksp. 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