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Although both 2-wk-old Cbs<jats:sup>\u2212\/\u2212<\/jats:sup>and Cth<jats:sup>\u2212\/\u2212<\/jats:sup>mice had normal renal architecture, their serum\/urinary amino acid profiles largely differed from wild-type mice. The most striking feature was marked accumulation of Met and cystathionine in serum\/urine\/kidney samples of Cbs<jats:sup>\u2212\/\u2212<\/jats:sup>and Cth<jats:sup>\u2212\/\u2212<\/jats:sup>mice, respectively. Levels of some neutral amino acids (Val, Leu, Ile, and Tyr) that were not elevated in Cbs<jats:sup>\u2212\/\u2212<\/jats:sup>serum were highly elevated in Cbs<jats:sup>\u2212\/\u2212<\/jats:sup>urine, and urinary excretion of other neutral amino acids (except Met) was much higher than expected from their serum levels, demonstrating neutral aminoaciduria in Cbs<jats:sup>\u2212\/\u2212<\/jats:sup>(not Cth<jats:sup>\u2212\/\u2212<\/jats:sup>) mice. Because the bulk of neutral amino acids is absorbed via a B<jats:sup>0<\/jats:sup>AT1 transporter and Met has the highest substrate affinity for B<jats:sup>0<\/jats:sup>AT1 than other neutral amino acids, hypermethioninemia may cause hyperexcretion of neutral amino acids.<\/jats:p>","DOI":"10.1152\/ajprenal.00623.2013","type":"journal-article","created":{"date-parts":[[2014,4,24]],"date-time":"2014-04-24T07:24:49Z","timestamp":1398324289000},"page":"F1462-F1476","source":"Crossref","is-referenced-by-count":15,"title":["Neutral aminoaciduria in cystathionine \u03b2-synthase-deficient mice, an animal model of homocystinuria"],"prefix":"10.1152","volume":"306","author":[{"given":"Noriyuki","family":"Akahoshi","sequence":"first","affiliation":[{"name":"Department of Molecular and Cellular Neurobiology, Gunma University Graduate School of Medicine, Gunma, Japan;"},{"name":"Japan Science and Technology Agency (JST), Exploratory Research for Advanced Technology (ERATO), Suematsu Gas Biology 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