Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria

Solverson, Patrick; Murali, Sangita G.; Brinkman, Adam S.; Nelson, David W.; Clayton, Murray K.; Yen, Chi‐Liang Eric; Ney, Denise M.

doi:10.1152/ajpendo.00647.2011

Cited by 49 publications

(60 citation statements)

References 53 publications

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“…Experimental animals were produced from a breeding colony of PKU mice by breeding C57BL/6J mice heterozygous for the Pah enu2 mutation to yield homozygous PKU mice and WT control mice [34,35]. Experimental mice were genotyped for the presence of the Pah enu2 mutation [36]. The experimental design was set to control for three main effects and their interactions; these being genotype (WT or PKU), sex (male or female), and diet (low-phe GMP, low-phe AA, or high-phe casein), in a 2×2×3 factorial design, Figure 1.…”

Section: Methodsmentioning

confidence: 99%

“…Mice were fed the experimental diet from weaning (3 weeks of age) through young adulthood, which resulted for an average feeding time of 18 ± 3 weeks (n = 284 mice). All the diets were isoenergetic with the protein source being the only source of variation (Harlan Teklad, Madison, WI; TD.09667 – TD.09669, TD.120645) [36]. The protein in the casein diet was provided by 20% (wt/wt) casein plus 0.3% L-cystine, the AA diet included 17.5% free AAs [37], and the GMP diet had 20% GMP (BioPURE GMP, Davisco Foods International, LeSueur, MN or LACPORDEN CGMP-10, Arla Foods Ingredients amba, Viby J, Denmark) plus 1.5 times the National Research Council (NRC) requirement for 5 limiting AA in order to provide a complete protein source.…”

Section: Methodsmentioning

confidence: 99%

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Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pahenu2 mice

Sawin

Murali

Ney

2014

Molecular Genetics and Metabolism

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Phenylketonuria (PKU) is an inborn error of metabolism caused by a deficiency of the enzyme phenylalanine hydroxylase, which metabolizes phenylalanine (phe) to tyrosine. A low-phe diet plus amino acid (AA) formula is necessary to prevent cognitive impairment; glycomacropeptide (GMP) contains minimal phe and provides a palatable alternative to the AA formula. Our objective was to assess neurotransmitter concentrations in brain and the behavioral phenotype of PKU mice (Pahenu2 on the C57Bl/6 background) and how this is affected by low-phe protein sources. Wild type (WT) and PKU mice, both male and female, were fed high-phe casein, low-phe AA, or low-phe GMP diets between 3–18 weeks of age. Behavioral phenotype was assessed using the open field and marble burying tests, and brain neurotransmitter concentration measured using HPLC with electrochemical detection system. Data were analyzed by 3-way ANOVA with genotype, sex, and diet as the main treatment effects. Brain mass and the concentrations of catecholamines and serotonin were reduced in PKU mice compared to WT mice; the low-phe AA and GMP diets improved these parameters in PKU mice. Relative brain mass was increased in female PKU mice fed the GMP diet compared to the AA diet. PKU mice exhibited hyperactivity and impaired vertical exploration compared to their WT littermates during the open field test. Regardless of genotype or diet, female mice demonstrated increased vertical activity time and increased total ambulatory and horizontal activity counts compared with male mice. PKU mice fed the high-phe casein diet buried significantly fewer marbles than WT control mice fed casein; this was normalized in PKU mice fed the low-phe AA and GMP diets. In summary, C57Bl/6-Pahenu2 mice showed an impaired behavioral phenotype and reduced brain neurotransmitter concentrations that were improved by the low-phe AA or GMP diets. These data support lifelong adherence to a low-phe diet for PKU.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pahenu2 mice

Sawin

Murali

Ney

2014

Molecular Genetics and Metabolism

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“…Energy expenditure, food intake, systemic inflammation markers and plasma level of phenylalanine were lowered more efficiently than that of casein diet (it has high phenylalanine content). Moreover, total fat mass and the respiratory exchange ratio were significantly lower in phenylketonuria mice fed with glycomacropeptide (Solverson et al 2012). Osteopenia (skeletal fragility due to reduced mineral content of bones) is a complication associated with phenylketonuria (Demirdas et al 2015).…”

Section: Phenylketonuria Managementmentioning

confidence: 99%

Emerging trends in nutraceutical applications of whey protein and its derivatives

Patel

2015

J Food Sci Technol

121

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The looming food insecurity demands the utilization of nutrient-rich residues from food industries as valueadded products. Whey, a dairy industry waste has been characterized to be excellent nourishment with an array of bioactive components. Whey protein comprises 20 % of total milk protein and it is rich in branched and essential amino acids, functional peptides, antioxidants and immunoglobulins. It confers benefits against a wide range of metabolic diseases such as cardiovascular complications, hypertension, obesity, diabetes, cancer and phenylketonuria. The protein has been validated to boost recovery from resistance exercise-injuries, stimulate gut physiology and protect skin against detrimental radiations. Apart from health invigoration, whey protein has proved its suitability as fat replacer and emulsifier. Further, its edible and antimicrobial packaging potential renders its highly desirable in food as well as pharmaceutical sectors. Considering the enormous nutraceutical worth of whey protein, this review emphasizes on its established and emerging biological roles. Present and future scopes in food processing and dietary supplement formulation are discussed. Associated hurdles are identified and how technical advancement might augment its applications are explored. This review is expected to provide valuable insight on whey protein-fortified functional foods, associated technical hurdles and scopes of improvement.

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“…It was reported that whey protein glycomacropeptide, owing to its low phenylalanine content, can be of immense therapeutic value to patients (Van Calcar & Ney, 2012). A study on a mouse model of phenylketonuria revealed that glycomacropeptide diet improves systemic inflammation markers (Solverson et al, 2012). Osteopenia characterized by bone mineral deficiency is a symptom of phenylketonuria.…”

Section: Phenylketonuria Therapymentioning

confidence: 99%

Functional food relevance of whey protein: A review of recent findings and scopes ahead

Patel

2015

Journal of Functional Foods

126

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Available online A B S T R A C TThe prospecting for nutrients has catapulted whey protein to the forefront of the functional food sector. This protein, filtered from cheese whey, has been characterized to contain a plethora of healthy components such as essential amino acids, bioactive peptides, antioxidants and immunomopotentiators. Consequently, whey protein has been validated to confer radical scavenging, anti-inflammatory, antitumour, immunostimulatory, hypotensive, gut homeostasis, antiobesity, antidiabetic, muscle biosynthesis, osteoprotective and radioprotective roles. As the multifarious benefits of whey protein cumulate amidst the dramatic rise in metabolic and degenerative health issues, it seems imperative to harness their potential. This review presents the significant biological aspects of whey protein and its derivatives. Further, it rationalizes their incorporation in functional foods.

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Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria

Cited by 49 publications

References 53 publications

Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pahenu2 mice

Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pahenu2 mice

Emerging trends in nutraceutical applications of whey protein and its derivatives

Functional food relevance of whey protein: A review of recent findings and scopes ahead

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