ArticlePloS one2012
Low bone strength is a manifestation of phenylketonuria in mice and is attenuated by a glycomacropeptide diet.
Article in PloS one, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
35 citing papers in PubMed, 1 synthesis or guideline pooled it, 58 citations in OpenAlex.
- The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis.Nutrients · 2018Pooled it
- Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial.The American journal of clinical nutrition · 2016Trial
- Whey Proteins and Immunity: Mechanisms Underlying Immune System Reinforcement and Protection Against Viral and Bacterial Infections.Nutrients · 2026Review
- Global Use of Casein Glycomacropeptide Protein Substitutes for Phenylketonuria (PKU): Health Professional Perspectives.Nutrients · 2026Article
- Whey Proteins and Bioactive Peptides: Advances in Production, Selection and Bioactivity Profiling.Biomedicines · 2025Review
- Preparing Enteral Formulas for Adult Patients with Phenylketonuria: A Minor Necessity but Major Challenge-A Case Report.Journal of clinical medicine · 2023Article
- Creatine energy substrate increases bone density in the PahMolecular genetics and metabolism reports · 2023Article
- Quality of life aspects of a low protein diet using GMP in patients with phenylketonuria.The Journal of international medical research · 2022Observational
- Phenylketonuria oxidative stress and energy dysregulation: Emerging pathophysiological elements provide interventional opportunity.Molecular genetics and metabolism · 2022Review
- Glycomacropeptide Safety and Its Effect on Gut Microbiota in Patients with Phenylketonuria: A Pilot Study.Nutrients · 2022Article
- Glycomacropeptide in PKU-Does It Live Up to Its Potential?Nutrients · 2022Review
- The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model.PloS one · 2022Article
- Article
- Mesenchymal stem cell energy deficit and oxidative stress contribute to osteopenia in the PahMolecular genetics and metabolism · 2021Article
- Nutrition, Microbiota and Role of Gut-Brain Axis in Subjects with Phenylketonuria (PKU): A Review.Nutrients · 2020Review
- Sex effects of dietary protein source and acid load on renal and bone status in the PahPhysiological reports · 2019Article
- Glycomacropeptide Bioactivity and Health: A Review Highlighting Action Mechanisms and Signaling Pathways.Nutrients · 2019Review
- Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU.Orphanet journal of rare diseases · 2019Review
- A bone mineralization defect in the PahMolecular genetics and metabolism · 2018Article
- Sex differences in body composition and bone mineral density in phenylketonuria: A cross-sectional study.Molecular genetics and metabolism reports · 2018Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
purposePhenylketonuria (PKU), caused by phenylalanine (phe) hydroxylase loss of function mutations, requires a low-phe diet plus amino acid (AA) formula to prevent cognitive impairment. Glycomacropeptide (GMP), a low-phe whey protein, provides a palatable alternative to AA formula. Skeletal fragility is a poorly understood chronic complication of PKU. We sought to characterize the impact of the PKU genotype and dietary protein source on bone biomechanics. PROCEDURES: Wild type (WT; Pah(+/+)) and PKU (Pah(enu2/enu2)) mice on a C57BL/6J background were fed high-phe casein, low-phe AA, and low-phe GMP diets between 3 to 23 weeks of age. Following euthanasia, femur biomechanics were assessed by 3-point bending and femoral diaphyseal structure was determined. Femoral ex vivo bone mineral density (BMD) was assessed by dual-energy x-ray absorptiometry. Whole bone parameters were used in principal component analysis. Data were analyzed by 3-way ANCOVA with genotype, sex, and diet as the main factors.
findingsRegardless of diet and sex, PKU femora were more brittle, as manifested by lower post-yield displacement, weaker, as manifested by lower energy and yield and maximal loads, and showed reduced BMD compared with WT femora. Four principal components accounted for 87% of the variance and all differed significantly by genotype. Regardless of genotype and sex, the AA diet reduced femoral cross-sectional area and consequent maximal load compared with the GMP diet.
conclusionsSkeletal fragility, as reflected in brittle and weak femora, is an inherent feature of PKU. This PKU bone phenotype is attenuated by a GMP diet compared with an AA diet.
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Registered trials
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