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Phenylketonuria: Protein content and amino acids profile of dishes for phenylketonuric patients. The relevance of phenylalanine

dc.contributor.authorPimentel, Filipa B.
dc.contributor.authorAlves, Rita C.
dc.contributor.authorCosta, Anabela S. G.
dc.contributor.authorTorres, Duarte
dc.contributor.authorAlmeida, Manuela F.
dc.contributor.authorOliveira, M. Beatriz P. P.
dc.date.accessioned2014-12-22T14:47:18Z
dc.date.available2014-12-22T14:47:18Z
dc.date.issued2014-04-15
dc.description.abstractPhenylketonuria is an inborn error of metabolism, involving, in most cases, a deficient activity of phenylalanine hydroxylase. Neonatal diagnosis and a prompt special diet (low phenylalanine and natural-protein restricted diets) are essential to the treatment. The lack of data concerning phenylalanine contents of processed foodstuffs is an additional limitation for an already very restrictive diet. Our goals were to quantify protein (Kjeldahl method) and amino acid (18) content (HPLC/fluorescence) in 16 dishes specifically conceived for phenylketonuric patients, and compare the most relevant results with those of several international food composition databases. As might be expected, all the meals contained low protein levels (0.67–3.15 g/100 g) with the highest ones occurring in boiled rice and potatoes. These foods also contained the highest amounts of phenylalanine (158.51 and 62.65 mg/100 g, respectively). In contrast to the other amino acids, it was possible to predict phenylalanine content based on protein alone. Slight deviations were observed when comparing results with the different food composition databases.por
dc.identifier.doi10.1016/j.foodchem.2013.10.099
dc.identifier.urihttp://hdl.handle.net/10400.22/5269
dc.language.isoengpor
dc.peerreviewedyespor
dc.publisherElsevierpor
dc.relation.ispartofseriesFood Chemistry;Vol. 149
dc.relation.publisherversionhttp://www.sciencedirect.com/science/article/pii/S0308814613015483por
dc.subjectPhenylketonuriapor
dc.subjectLow protein foodspor
dc.subjectProtein contentpor
dc.subjectAmino acidspor
dc.subjectPhenylalaninepor
dc.titlePhenylketonuria: Protein content and amino acids profile of dishes for phenylketonuric patients. The relevance of phenylalaninepor
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage150por
oaire.citation.startPage144por
oaire.citation.titleFood Chemistrypor
oaire.citation.volume149por
person.familyNameAlves
person.givenNameRita C.
person.identifier.orcid0000-0002-5053-513X
person.identifier.ridI-9081-2012
person.identifier.scopus-author-id7102691286
rcaap.rightsopenAccesspor
rcaap.typearticlepor
relation.isAuthorOfPublication4b5cf5a1-8445-4d6a-ac30-5fbee0326269
relation.isAuthorOfPublication.latestForDiscovery4b5cf5a1-8445-4d6a-ac30-5fbee0326269

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