Dietary interventions for phenylketonuria

Poustie, Vanessa; Wildgoose, Joanne; Rutherford, Patricia

doi:10.1002/14651858.cd001304

Cited by 20 publications

(22 citation statements)

References 31 publications

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“…Both hydrolysates showed an amount of phenylalanine around 15 mg/100 g of protein, which means that it could be used in food products to phenylketonuric patients. The products for phenylketonuric patients must have a lower phenylalanine concentration (10-20 mg/100 g of product) (Poustie and Rutherford 2002).…”

Section: Emulsifying Activity Index (Eai) and Emulsion Stability (Es)mentioning

confidence: 99%

Evaluation of Functional Properties in Protein Hydrolysates from Bluewing Searobin (Prionotus punctatus) Obtained with Different Microbial Enzymes

Santos

Martins

Salas-Mellado

et al. 2009

Food Bioprocess Technol

101

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Enzymatic hydrolysis of proteins from low commercial value fish could be produced for uses like functional ingredients in a wide and always increasing zone of application in different food products. The objective of this work was to evaluate the functional properties and the amino acid profile of enzymatic hydrolysates from Bluewing searobin (Prionotus punctatus), using two microbial enzymes, Alcalase and Flavourzyme. The enzymatic hydrolysate obtained through the addition of the enzyme Alcalase reached the maximum solubility (42%) at pH 9, water holding capacity (WHC) of 2.4 g water g protein −1 , 4.5 g oil g protein −1 of oil holding capacity (OHC) and an emulsifying activity index (EAI) of 54 m 2 g solids −1 at pH 3. On the other hand, the hydrolysate obtained from Flavourzyme attained 38% of solubility at pH 9, 3.7 g water g protein −1 and 5.5 g oil g protein −1 for the holding capacities, and an EAI of 71 m 2 g solids −1 at pH 11. The hydrolysate with Flavourzyme produced best results for WHC, OHC, and EAI because it had solubility lower than the hydrolysate of Alcalase. The hydrolysate produced by Alcalase had a higher amino acid content compared with Flavourzyme's hydrolysate. However, both showed a good essential amino acid amounts. In general, these results indicate the potential utilization of the hydrolysate from Bluewing searobin in food formulations for the direct human consumption.

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Section: Emulsifying Activity Index (Eai) and Emulsion Stability (Es)mentioning

confidence: 99%

Evaluation of Functional Properties in Protein Hydrolysates from Bluewing Searobin (Prionotus punctatus) Obtained with Different Microbial Enzymes

Santos

Martins

Salas-Mellado

et al. 2009

Food Bioprocess Technol

101

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“…Treatment for PKU patients consists of restriction of Phe intake, through natural-protein-restricted diets supplemented with phenylalanine-free amino acid mixtures enriched with trace elements, vitamins, and minerals (Przyrembel and Bremer 2000;Giovannini et al 2007;Poustie and Wildgoose 2010). When the disorder is diagnosed and treated promptly at an early age, mental retardation can be prevented, although mild neuropsychological findings, such as poor school performance and a slight reduction in the intelligence quotient may occur, especially when careful dietary compliance is not achieved (Weglage et al 1995).…”

Section: Introductionmentioning

confidence: 99%

Oxidative Stress in Phenylketonuria: What is the Evidence?

et al. 2011

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Phenylketonuria (PKU) is an inborn error of amino acid metabolism caused by severe deficiency of phenylalanine hydroxylase activity, leading to the accumulation of phenylalanine and its metabolites in blood and tissues of affected patients. Phenylketonuric patients present as the major clinical feature mental retardation, whose pathomechanisms are poorly understood. In recent years, mounting evidence has emerged indicating that oxidative stress is possibly involved in the pathology of PKU. This article addresses some of the recent developments obtained from animal studies and from phenylketonuric patients indicating that oxidative stress may represent an important element in the pathophysiology of PKU. Several studies have shown that enzymatic and non-enzymatic antioxidant defenses are decreased in plasma and erythrocytes of PKU patients, which may be due to an increased free radical generation or secondary to the deprivation of micronutrients which are essential for these defenses. Indeed, markers of lipid, protein, and DNA oxidative damage have been reported in PKU patients, implying that reactive species production is increased in this disorder. A considerable set of data from in vitro and in vivo animal studies have shown that phenylalanine and/or its metabolites elicit reactive species in brain rodent. These findings point to a disruption of pro-oxidant/antioxidant balance in PKU. Considering that the brain is particularly vulnerable to oxidative attack, it is presumed that the administration of appropriate antioxidants as adjuvant agents, in addition to the usual treatment based on restricted diets or supplementation of tetrahydrobiopterin, may represent another step in the prevention of the neurological damage in PKU.

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“…The subsequent elevation of serum phenylalanine is toxic to the developing central nervous system of the child, resulting in irreversible neurological damage (Bickel et al, 1954). Early detection and treatment with dietary phenylalanine restriction from the first weeks of life to maintain serum phenylalanine within controlled ranges allows normal cognitive development (Poustie & Rutherford, 2001). The introduction of national routine neonatal screening (Guthrie test) within the last 40 years means there is now a 'first generation' of young adults with PKU living independent lives.…”

Section: Introductionmentioning

confidence: 99%