Page last updated: 2024-09-04

caseins and BH4 Deficiency

caseins has been researched along with BH4 Deficiency in 36 studies

Research

Studies (36)

Timeframe	Studies, this research(%)	All Research%
pre-1990	5 (13.89)	18.7374
1990's	0 (0.00)	18.2507
2000's	0 (0.00)	29.6817
2010's	19 (52.78)	24.3611
2020's	12 (33.33)	2.80

Authors

Authors	Studies
Ahring, KK; Brüel, A; Brøndum-Nielsen, K; Christensen, M; Dagnæs-Hansen, F; Jensen, E; Jensen, TG; Johannsen, M; Johansen, KS; Kjolby, M; Lund, AM; Madsen, JG; Møller, LB; Pedersen, M; Sørensen, LK	1
Daly, A; Evans, S; MacDonald, A; Pinto, A	1
Bighinati, A; Giardino, L; Giarratana, N; Reiner, G; Rocha, JC	1
Banderali, G; Borghi, E; Ceccarani, C; Corsello, A; Dei Cas, M; Montanari, C; Ottaviano, E; Verduci, E; Zuccotti, G; Zuvadelli, J	1
Bensi, G; Biasucci, G; Carbone, MT; Parolisi, S; Pozzoli, A; Schiaffino, MC	1
Bollati, C; Burlina, A; d'Adduzio, L; Fanzaga, M; Fiori, L; Lammi, C; Li, J; Tosi, M; Verduci, E; Zuccotti, G	1
Ashmore, C; Covington, JA; Daly, A; Evans, S; Hattersley, J; MacDonald, A; Pinto, A; Tiele, A	1
Ashmore, C; Daly, A; Evans, S; Jackson, R; MacDonald, A; Pinto, A; Rocha, JC	2
Ashmore, C; Daly, A; Evans, S; MacDonald, A; Pinto, A	1
Bandeira, A; Borges, N; de Almeida, MF; de Sousa Barbosa, C; Dias, CC; Guimas, A; MacDonald, A; Martins, E; Pena, MJ; Pinto, A; Ramos, PC; Ribeiro, R; Rocha, JC; Rocha, S	1
Ashmore, C; Crabtree, N; Daly, A; Evans, S; Högler, W; Jackson, R; MacDonald, A; Pinto, A; Rocha, JC; Shaw, N; Strauss, BJ; Wilcox, G	1
Levy, HL; Murali, SG; Nair, N; Ney, DM; Rohr, F; Sawin, EA; Stroup, BM	1
Almeida, MF; Bandeira, A; Guimas, A; MacDonald, A; Martins, E; Pinto, A; Ramos, PC; Ribeiro, R; Rocha, JC; Rocha, S	1
Broniowska, K; Levy, HL; Murali, SG; Nair, N; Ney, DM; Rohr, F; Stroup, BM	1
Dotsikas, Y; Loukas, YL; Manta-Vogli, PD; Schulpis, KH	1
Azevedo, L; Borges, N; Daly, A; MacDonald, A; Pena, MJ; Pinto, A; Rocha, JC	1
Chahal, S; Daly, A; Evans, S; Gingell, C; Jackson, R; MacDonald, A; Pinto, A; Rocha, J; Santra, S; Van Spronsen, FJ	1
Azevedo, TD; Cândido, LMB; Krüger, CCH; Piltz, MT; Silva, ÁT	1
Bonnemains, C; Feillet, F	1
Blank, RD; Hansen, KE; Ney, DM	1
Murali, SG; Ney, DM; Sawin, EA	1
Aguiar, A; Ahring, K; Almeida, MF; Assoun, M; Belanger Quintana, A; Bigot, S; Bihet, G; Blom Malmberg, K; Burlina, A; Bushueva, T; Caris, A; Chan, H; Clark, A; Clark, S; Cochrane, B; Corthouts, K; Dalmau, J; Dassy, M; De Meyer, A; Didycz, B; Diels, M; Dokupil, K; Dubois, S; Eftring, K; Ekengren, J; Ellerton, C; Evans, S; Faria, A; Fischer, A; Ford, S; Freisinger, P; Giżewska, M; Gokmen-Ozel, H; Gribben, J; Gunden, F; Heddrich-Ellerbrok, M; Heiber, S; Heidenborg, C; Jankowski, C; Janssen-Regelink, R; Joerg-Streller, M; Jones, I; Jonkers, C; Kaalund-Hansen, K; Kiss, E; Lammardo, AM; Lang, K; Lier, D; Lilje, R; Lowry, S; Luyten, K; MacDonald, A; Meyer, U; Moor, D; Pal, A; Robert, M; Robertson, L; Rocha, JC; Rohde, C; Ross, K; Saruhan, S; Sjöqvist, E; Skeath, R; Stoelen, L; Ter Horst, NM; Terry, A; Timmer, C; Tuncer, N; van der Ploeg, L; van Rijn, M; van Spronsen, FJ; van Teeffelen-Heithoff, A; van Wegberg, A; van Wyk, K; Vande Kerckhove, K; Vasconcelos, C; Vitoria, I; Webster, D; White, FJ; Wildgoose, J; Zweers, H	1
Adachi, O; Castañeda, MT; Hours, RA	1
Aktas, B; De Wolfe, TJ; Murali, SG; Ney, DM; Sawin, EA; Steele, JL; Stroup, BM	1
Ahring, K; Bélanger-Quintana, A; de Almeida, MF; Dokoupil, K; Gokmen-Ozel, H; Lammardo, AM; MacDonald, A; Pena, MJ; Robert, M; Rocha, JC; van Dam, E	1
Clayton, MK; Levy, HL; Murali, SG; Ney, DM; Rice, GM; Rohr, F; Stroup, BM	1
Chahal, S; Daly, A; Evans, S; MacDonald, A; Santra, S	1
Clayton, MK; MacLeod, EL; Ney, DM; van Calcar, SC	1
Brinkman, AS; Clayton, MK; Murali, SG; Nelson, DW; Ney, DM; Solverson, P; Yen, CL	1
Blank, RD; Litscher, SJ; Murali, SG; Ney, DM; Solverson, P	1
GRUTTNER, R; MULLER, F; WALLIS, H	1
O'DALY, S	1
PAINE, RS	1
BOELENS, CH	1

Reviews

8 review(s) available for caseins and BH4 Deficiency

Article	Year
Glycomacropeptide in PKU-Does It Live Up to Its Potential? Nutrients, 2022, Feb-14, Volume: 14, Issue:4 Topics: Animals; Caseins; Peptide Fragments; Phenylalanine; Phenylketonurias	2022
Protein Substitutes in PKU; Their Historical Evolution. Nutrients, 2021, Feb-02, Volume: 13, Issue:2 Topics: Amino Acids; Animals; Caseins; Child, Preschool; Dietary Proteins; Female; History, 20th Century; History, 21st Century; Humans; Nutritional Requirements; Peptide Fragments; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids; Protein Hydrolysates; United Kingdom	2021
The phenylketonuria patient: A recent dietetic therapeutic approach. Nutritional neuroscience, 2020, Volume: 23, Issue:8 Topics: Amino Acids; Animals; Biopterins; Caseins; Diet; Diet, Protein-Restricted; Dietetics; Humans; Peptide Fragments; Phenylketonurias	2020
The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis. Nutrients, 2018, Nov-18, Volume: 10, Issue:11 Topics: Amino Acids; Caseins; Diet, Protein-Restricted; Dietary Supplements; Humans; Peptide Fragments; Phenylalanine; Phenylketonurias; Tyrosine	2018
Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU. Orphanet journal of rare diseases, 2019, 02-15, Volume: 14, Issue:1 Topics: Adolescent; Caseins; Child; Child, Preschool; Female; Humans; Longitudinal Studies; Male; Nutritional Status; Peptide Fragments; Phenylalanine; Phenylketonurias	2019
[Phenylketonuria: new treatments]. Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, 2013, Volume: 20, Issue:10 Topics: Amino Acids, Neutral; Biopterins; Caseins; Dietary Supplements; Enzyme Replacement Therapy; Genetic Therapy; Humans; Peptide Fragments; Phenylalanine Ammonia-Lyase; Phenylketonurias	2013
Advances in the nutritional and pharmacological management of phenylketonuria. Current opinion in clinical nutrition and metabolic care, 2014, Volume: 17, Issue:1 Topics: Amino Acids; Biopterins; Bone and Bones; Caseins; Dietary Supplements; Humans; Milk Proteins; Mutation; Peptide Fragments; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Randomized Controlled Trials as Topic; Tyrosine; Whey Proteins	2014
Amino acid intoxication. Food and cosmetics toxicology, 1971, Volume: 9, Issue:1 Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Benzyl Compounds; Biological Transport, Active; Brain; Caseins; Chickens; Cytoplasm; Endoplasmic Reticulum; Fasting; Feeding Behavior; Glutathione; Growth; Humans; Injections, Intraperitoneal; Leucine; Liver; Methionine; Microsomes; Mitochondrial Swelling; Phenylalanine; Phenylketonurias; Phenylpyruvic Acids; Protein Biosynthesis; Rats; Tyrosine	1971

Trials

4 trial(s) available for caseins and BH4 Deficiency

Article	Year
Metabolomic changes demonstrate reduced bioavailability of tyrosine and altered metabolism of tryptophan via the kynurenine pathway with ingestion of medical foods in phenylketonuria. Molecular genetics and metabolism, 2017, Volume: 121, Issue:2 Topics: Adolescent; Adult; Amino Acids; Biological Availability; Caseins; Catecholamines; Cross-Over Studies; Diet; Female; Food, Formulated; Gastrointestinal Microbiome; Humans; Kynurenine; Male; Melatonin; Metabolic Networks and Pathways; Metabolomics; Middle Aged; Peptide Fragments; Phenylketonurias; Prebiotics; Serotonin; Tryptophan; Tyrosine; Young Adult	2017
Metabolomic Markers of Essential Fatty Acids, Carnitine, and Cholesterol Metabolism in Adults and Adolescents with Phenylketonuria. The Journal of nutrition, 2018, 02-01, Volume: 148, Issue:2 Topics: Adolescent; Adult; Amino Acids; Betaine; Biomarkers; Carnitine; Caseins; Cholesterol; Cross-Over Studies; Dietary Supplements; Erythrocytes; Fasting; Fatty Acids; Fatty Acids, Essential; Female; Gastrointestinal Microbiome; Humans; Lipid Metabolism; Male; Metabolomics; Methylamines; Middle Aged; Peptide Fragments; Phenylketonurias	2018
Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial. The American journal of clinical nutrition, 2016, Volume: 104, Issue:2 Topics: Adolescent; Adult; Analysis of Variance; Caseins; Cross-Over Studies; Dietary Proteins; Feeding Behavior; Female; Foods, Specialized; Gastrointestinal Diseases; Humans; Hunger; Male; Middle Aged; Patient Satisfaction; Peptide Fragments; Phenylalanine; Phenylketonurias; Young Adult	2016
Breakfast with glycomacropeptide compared with amino acids suppresses plasma ghrelin levels in individuals with phenylketonuria. Molecular genetics and metabolism, 2010, Volume: 100, Issue:4 Topics: Adolescent; Adult; Amino Acids; Caseins; Child; Diet; Female; Food; Ghrelin; Humans; Insulin; Male; Motivation; Pain Measurement; Peptide Fragments; Phenylketonurias; Postprandial Period; Satiety Response; Young Adult	2010

Other Studies

24 other study(ies) available for caseins and BH4 Deficiency

Article	Year
The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model. PloS one, 2022, Volume: 17, Issue:1 Topics: Amino Acids; Animals; Bone and Bones; Bone Density; Brain; Caseins; Dietary Supplements; Disease Models, Animal; Female; Maze Learning; Mice; Mice, Inbred C57BL; Peptide Fragments; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias; Serotonin; Tyrosine	2022
In Vivo Metabolic Responses to Different Formulations of Amino Acid Mixtures for the Treatment of Phenylketonuria (PKU). International journal of molecular sciences, 2022, Feb-17, Volume: 23, Issue:4 Topics: Amino Acids; Animals; Biomarkers; Caseins; Diet, Protein-Restricted; Male; Membrane Proteins; Myostatin; Phenylketonurias; Rats; Rats, Wistar	2022
Glycomacropeptide Safety and Its Effect on Gut Microbiota in Patients with Phenylketonuria: A Pilot Study. Nutrients, 2022, Apr-29, Volume: 14, Issue:9 Topics: Animals; Caseins; Gastrointestinal Microbiome; Humans; Mice; Peptide Fragments; Phenylketonurias; Pilot Projects; Prebiotics; RNA, Ribosomal, 16S	2022
Quality of life aspects of a low protein diet using GMP in patients with phenylketonuria. The Journal of international medical research, 2022, Volume: 50, Issue:9 Topics: Caseins; Cholesterol; Diet, Protein-Restricted; Humans; Peptide Fragments; Phenylketonurias; Quality of Life; Vitamins	2022
Glycomacropeptide (GMP) rescued the oxidative and inflammatory activity of free L-AAs in human Caco-2 cells: New insights that support GMP as a valid and health-promoting product for the dietary management of phenylketonuria (PKU) patients. Food research international (Ottawa, Ont.), 2023, Volume: 173, Issue:Pt 1 Topics: Caco-2 Cells; Caseins; Humans; Hydrogen Peroxide; Phenylketonurias	2023
Investigation of paediatric PKU breath malodour, comparing glycomacropeptide with phenylalanine free L-amino acid supplements. Journal of breath research, 2019, 10-21, Volume: 14, Issue:1 Topics: Adolescent; Breath Tests; Caseins; Child; Confounding Factors, Epidemiologic; Cross-Over Studies; Dietary Supplements; Exhalation; Female; Gas Chromatography-Mass Spectrometry; Humans; Longitudinal Studies; Male; Peptide Fragments; Phenylalanine; Phenylketonurias; Surveys and Questionnaires; Volatile Organic Compounds	2019
Preliminary Investigation to Review If a Glycomacropeptide Compared to L-Amino Acid Protein Substitute Alters the Pre- and Postprandial Amino Acid Profile in Children with Phenylketonuria. Nutrients, 2020, Aug-14, Volume: 12, Issue:8 Topics: Adolescent; Age Factors; Amino Acids; Caseins; Child; Child, Preschool; Female; Humans; Insulin; Male; Nitrogen; Peptide Fragments; Phenylalanine; Phenylketonurias; Postprandial Period; Time Factors; Urea	2020
The Impact of the Use of Glycomacropeptide on Satiety and Dietary Intake in Phenylketonuria. Nutrients, 2020, Sep-04, Volume: 12, Issue:9 Topics: Adolescent; Amino Acids; Anthropometry; Body Mass Index; Body Weight; Caseins; Child; Child, Preschool; Diet Surveys; Diet, Protein-Restricted; Energy Intake; Female; Glycopeptides; Humans; Longitudinal Studies; Male; Nutrients; Phenylalanine; Phenylketonurias; Prospective Studies; Satiation	2020
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective. Orphanet journal of rare diseases, 2021, 02-13, Volume: 16, Issue:1 Topics: Adolescent; Adult; Caseins; Female; Humans; Male; Peptide Fragments; Phenylketonurias; Prospective Studies; Retrospective Studies; Young Adult	2021
Growth and Body Composition in PKU Children-A Three-Year Prospective Study Comparing the Effects of L-Amino Acid to Glycomacropeptide Protein Substitutes. Nutrients, 2021, Apr-16, Volume: 13, Issue:4 Topics: Absorptiometry, Photon; Adolescent; Amino Acids; Body Composition; Body Height; Caseins; Child; Child, Preschool; Dietary Proteins; Dietary Supplements; Female; Growth; Humans; Longitudinal Studies; Male; Peptide Fragments; Phenylalanine; Phenylketonurias; Prospective Studies; Treatment Outcome	2021
Nutritional status in patients with phenylketonuria using glycomacropeptide as their major protein source. European journal of clinical nutrition, 2017, Volume: 71, Issue:10 Topics: Adolescent; Adult; Body Composition; Caseins; Dietary Proteins; Female; Humans; Longitudinal Studies; Male; Middle Aged; Nutritional Status; Peptide Fragments; Phenylketonurias; Portugal; Retrospective Studies; Young Adult	2017
Casein-derived peptides as an alternative ingredient for low-phenylalanine diets. Nutricion hospitalaria, 2019, Jul-01, Volume: 36, Issue:3 Topics: Amino Acids; Caseins; Diet; Humans; Peptides; Phenylalanine; Phenylketonurias	2019
Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pah(enu2) mice. Molecular genetics and metabolism, 2014, Volume: 111, Issue:4 Topics: Animals; Behavior, Animal; Brain; Caseins; Catecholamines; Dietary Proteins; Female; Genotype; Male; Mice; Mice, Inbred C57BL; Motor Activity; Neurotransmitter Agents; Organ Size; Peptide Fragments; Phenylalanine; Phenylketonurias; Serotonin; Time Factors	2014
Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach. Molecular genetics and metabolism, 2015, Volume: 115, Issue:1 Topics: Adult; Amino Acids; Caseins; Child; Child, Preschool; Dietary Proteins; Dietary Supplements; Europe; Female; Humans; Infant; Infant, Newborn; Male; Peptide Fragments; Phenylalanine; Phenylketonurias; Surveys and Questionnaires; Turkey; World Health Organization	2015
Reduction of L-phenylalanine in protein hydrolysates using L-phenylalanine ammonia-lyase from Rhodosporidium toruloides. Journal of industrial microbiology & biotechnology, 2015, Volume: 42, Issue:10 Topics: Basidiomycota; Caseins; Cinnamates; Dietary Supplements; Humans; Hydrogen-Ion Concentration; Kinetics; Phenylalanine; Phenylalanine Ammonia-Lyase; Phenylketonurias; Protein Hydrolysates; Temperature	2015
Glycomacropeptide is a prebiotic that reduces Desulfovibrio bacteria, increases cecal short-chain fatty acids, and is anti-inflammatory in mice. American journal of physiology. Gastrointestinal and liver physiology, 2015, Oct-01, Volume: 309, Issue:7 Topics: Animals; Caseins; Cecum; Cytokines; Desulfovibrio; Fatty Acids, Volatile; Feces; Female; Flow Cytometry; Gastrointestinal Microbiome; Male; Mice; Mice, Inbred C57BL; Peptide Fragments; Phenylketonurias; Prebiotics	2015
Protein substitutes for phenylketonuria in Europe: access and nutritional composition. European journal of clinical nutrition, 2016, Volume: 70, Issue:7 Topics: Amino Acids; Amino Acids, Neutral; Caseins; Dietary Proteins; Europe; Food, Formulated; Humans; Peptide Fragments; Phenylalanine; Phenylketonurias; Turkey	2016
Glycomacropeptide in children with phenylketonuria: does its phenylalanine content affect blood phenylalanine control? Journal of human nutrition and dietetics : the official journal of the British Dietetic Association, 2017, Volume: 30, Issue:4 Topics: Adolescent; Amino Acids; Anthropometry; Caseins; Child; Diet; Female; Humans; Male; Micronutrients; Nutrition Assessment; Peptide Fragments; Phenylalanine; Phenylketonurias; Pilot Projects; Prospective Studies; Tyrosine	2017
Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria. American journal of physiology. Endocrinology and metabolism, 2012, Apr-01, Volume: 302, Issue:7 Topics: Absorptiometry, Photon; Amino Acids; Animals; Body Composition; Body Weight; Caseins; Cheese; Cytokines; Diet; Diet, Protein-Restricted; Energy Metabolism; Growth; Mice; Mice, Inbred C57BL; Organ Size; Oxygen Consumption; Peptide Fragments; Phenotype; Phenylketonurias; Spleen; Splenomegaly	2012
Low bone strength is a manifestation of phenylketonuria in mice and is attenuated by a glycomacropeptide diet. PloS one, 2012, Volume: 7, Issue:9 Topics: Absorptiometry, Photon; Animals; Biomechanical Phenomena; Bone Density; Caseins; Diet, Protein-Restricted; Elastic Modulus; Female; Femur; Male; Mice; Mice, Inbred C57BL; Mice, Transgenic; Peptide Fragments; Phenotype; Phenylalanine; Phenylalanine Hydroxylase; Phenylketonurias	2012
[Evaluation of the success of dietetic treatment with casein hydrolysate low in phenylalanine in phenylpyruvic oligophrenia]. Monatsschrift fur Kinderheilkunde, 1958, Volume: 106, Issue:2 Topics: Caseins; Dietetics; Humans; Phenylalanine; Phenylketonurias	1958
Phenylketonuria treated with a high phenylalanine intake and casein-hydrolysate/aminoacid mixtures. Lancet (London, England), 1961, Jun-24, Volume: 1, Issue:7191 Topics: Amino Acids; Caseins; Diet; Humans; Nutrition Assessment; Nutritional Status; Phenylalanine; Phenylketonurias	1961
PHENYLKETONURIA. Clinical proceedings - Children's Hospital of the District of Columbia, 1964, Volume: 20 Topics: Body Fluids; Caseins; Child; Diet; Diet Therapy; Eczema; Humans; Infant; Infant, Newborn; Intellectual Disability; Metabolism; Microcephaly; Phenylalanine; Phenylketonurias; Urine	1964
[MANAGEMENT OF PATIENTS WITH PHENYLKETONURIA]. Maandschrift voor kindergeneeskunde, 1964, Volume: 32 Topics: Blood; Caseins; Child; Diagnosis; Diet; Diet Therapy; Disease Management; Humans; Infant; Infant, Newborn; Phenylalanine; Phenylketonurias	1964