{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T15:42:06Z","timestamp":1790696526209,"version":"4.1.0"},"reference-count":76,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2009,5,13]],"date-time":"2009-05-13T00:00:00Z","timestamp":1242172800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2009,8]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>Phenylketonuria is an inborn error of amino acid metabolism that results in severe mental retardation if not treated early and appropriately. The traditional treatment, consisting of a low\u2010phenylalanine diet, is usually difficult to maintain throughout adolescence and adulthood, resulting in undesirable levels of blood phenylalanine and consequent neurotoxicity. The neurotoxicity of phenylalanine is enhanced by its transport mechanism across the blood\u2010brain barrier, which has the highest affinity for phenylalanine compared with the other large neutral amino acids that share the same carrier. The supplementation of large neutral amino acids in phenylketonuric patients has been showing interesting results. Plasma phenylalanine levels can be reduced, which may guarantee important metabolic and clinical benefits to these patients. Although long\u2010term studies are needed to determine the efficacy and safety of large neutral amino acids supplements, the present state of knowledge seems to recommend their prescription to all phenylketonuric adult patients who are non\u2010compliant with the low\u2010phenylalanine diet.<\/jats:p>","DOI":"10.1007\/s10545-009-1132-x","type":"journal-article","created":{"date-parts":[[2009,5,12]],"date-time":"2009-05-12T02:19:59Z","timestamp":1242094799000},"page":"472-480","source":"Crossref","is-referenced-by-count":32,"title":["Large neutral amino acids supplementation in phenylketonuric patients"],"prefix":"10.1002","volume":"32","author":[{"given":"J. C.","family":"Rocha","sequence":"first","affiliation":[{"name":"Center of Medical Genetics Jacinto Magalh\u00e3es \u2014 INSA Porto Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Martel","sequence":"additional","affiliation":[{"name":"Department of Biochemistry (U38\u2010FCT), Faculty of Medicine of Porto University of Porto Al. Prof. Hern\u00e2ni Monteiro Porto 4200\u2010319 Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,5,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/130.11.2780"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198209000-00009"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(53)90473-5"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2060407"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/01376517-200008000-00008"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.40.3.648"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.00018.2006"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:BOLI.0000042985.02049.ff"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199703000-00011"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-008-0947-1"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005694122277"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.105.1.89"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bi.22.070153.001313"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejps.2008.06.015"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00711746"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.178.4059.414"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1010546000617"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00710427"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2788.1995.tb00540.x"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799701"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2004.03.042"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/136.1.218S"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2736(90)90261-L"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2008.12.019"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1059547"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.37.23629"},{"key":"e_1_2_7_28_1","first-page":"G501","article-title":"Uptake pathways for amino acids in mouse intestine","volume":"251","author":"Karasov W","year":"1986","journal-title":"Am J Physiol"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02436753"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.106.5.1136"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.106.5.1093"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1096-7192(03)00078-7"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1006\/jmrb.1995.1084"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70138-1"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.32562"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2007.09.017"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1994.tb13025.x"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1038\/26246"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1097\/00008486-200116040-00006"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-006-0395-8"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0556-4"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(72)90653-1"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1968.tb06202.x"},{"key":"e_1_2_7_44_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005638627604"},{"key":"e_1_2_7_45_1","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1542\/peds.112.S4.1575","article-title":"Brain phenylalanine concentrations in phenylketonuria: research and treatment of adults","volume":"112","author":"Moats RA","year":"2003","journal-title":"Pediatrics"},{"key":"e_1_2_7_46_1","doi-asserted-by":"publisher","DOI":"10.1007\/PL00014374"},{"key":"e_1_2_7_47_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1994.sp020344"},{"key":"e_1_2_7_48_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-008-0952-4"},{"key":"e_1_2_7_49_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199502000-00020"},{"key":"e_1_2_7_50_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00193.2003"},{"key":"e_1_2_7_51_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00187.2004"},{"key":"e_1_2_7_52_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11095-007-9374-5"},{"key":"e_1_2_7_53_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1022482604276"},{"key":"e_1_2_7_54_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199511000-00005"},{"key":"e_1_2_7_55_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004310050945"},{"key":"e_1_2_7_56_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI5017"},{"key":"e_1_2_7_57_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-200004270-00042"},{"key":"e_1_2_7_58_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(94)70360-4"},{"key":"e_1_2_7_59_1","first-page":"44","article-title":"Consensus for the nutritional treatment of phenylketonuria","volume":"38","author":"Rocha JC","year":"2007","journal-title":"Acta Pediatr Port"},{"key":"e_1_2_7_60_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)35701-6"},{"key":"e_1_2_7_61_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.25.14913"},{"key":"e_1_2_7_62_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-008-0917-7"},{"key":"e_1_2_7_63_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2007.02.002"},{"key":"e_1_2_7_64_1","doi-asserted-by":"publisher","DOI":"10.1080\/01688639408402681"},{"key":"e_1_2_7_65_1","first-page":"1667","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Scriver CR","year":"2001"},{"key":"e_1_2_7_66_1","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/130.4.1016S"},{"key":"e_1_2_7_67_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01868496"},{"key":"e_1_2_7_68_1","doi-asserted-by":"publisher","DOI":"10.1007\/PL00014370"},{"key":"e_1_2_7_69_1","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(90)93401-A"},{"key":"e_1_2_7_70_1","doi-asserted-by":"publisher","DOI":"10.3945\/ajcn.2008.27280"},{"key":"e_1_2_7_71_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1986.tb04382.x"},{"key":"e_1_2_7_72_1","doi-asserted-by":"publisher","DOI":"10.1038\/322639a0"},{"key":"e_1_2_7_73_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-008-0946-2"},{"key":"e_1_2_7_74_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(02)09334-0"},{"key":"e_1_2_7_75_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-540-28785-8_17"},{"key":"e_1_2_7_76_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.1226"},{"key":"e_1_2_7_77_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1021234730512"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1132-x","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1007\/s10545-009-1132-x","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-009-1132-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T14:32:40Z","timestamp":1694788360000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-009-1132-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,5,13]]},"references-count":76,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2009,8]]}},"alternative-id":["10.1007\/s10545-009-1132-x"],"URL":"https:\/\/doi.org\/10.1007\/s10545-009-1132-x","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,5,13]]}}}