ArticlePloS one2015
High Protein Diet and Huntington's Disease.
Article in PloS one, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 15 citations in OpenAlex.
- A Review of the Clinical Evidence for Complementary and Alternative Medicine in Huntington's Disease.Tremor and other hyperkinetic movements (New York, N.Y.) · 2019Pooled it
- Management and Prevention of Neurodegenerative Disorders: Can Antioxidant-Rich Dietary Interventions Help?Antioxidants (Basel, Switzerland) · 2025Review
- Metabolomics: a new frontier in neurodegenerative disease biomarker discovery.Metabolomics : Official journal of the Metabolomic Society · 2025Review
- Influence of dietary patterns in the pathophysiology of Huntington's Disease: A literature review.AIMS neuroscience · 2024Review
- Metabolomics: An Emerging "Omics" Platform for Systems Biology and Its Implications for Huntington Disease Research.Metabolites · 2023Review
- Glial Glutamine Homeostasis in Health and Disease.Neurochemical research · 2023Review
- Cross-sectional analysis of plasma and CSF metabolomic markers in Huntington's disease for participants of varying functional disability: a pilot study.Scientific reports · 2020Article
- Dietary Intake, Mediterranean Diet Adherence and Caloric Intake in Huntington's Disease: A Review.Nutrients · 2020Review
- Effects of a high protein diet and liver disease in an in silico model of human ammonia metabolism.Theoretical biology & medical modelling · 2019Article
- A Critical Evaluation of Wet Biomarkers for Huntington's Disease: Current Status and Ways Forward.Journal of Huntington's disease · 2018Review
- Brain urea increase is an early Huntington's disease pathogenic event observed in a prodromal transgenic sheep model and HD cases.Proceedings of the National Academy of Sciences of the United States of America · 2017Article
- Metabolic profiling of presymptomatic Huntington's disease sheep reveals novel biomarkers.Scientific reports · 2017Article
Corrections and comments
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Authors and funding
11 authors at 6 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Huntington's disease (HD) is a neurodegenerative disorder caused by the huntingtin (HTT) gene with expanded CAG repeats. In addition to the apparent brain abnormalities, impairments also occur in peripheral tissues. We previously reported that mutant Huntingtin (mHTT) exists in the liver and causes urea cycle deficiency. A low protein diet (17%) restores urea cycle activity and ameliorates symptoms in HD model mice. It remains unknown whether the dietary protein content should be monitored closely in HD patients because the normal protein consumption is lower in humans (~15% of total calories) than in mice (~22%). We assessed whether dietary protein content affects the urea cycle in HD patients. Thirty HD patients were hospitalized and received a standard protein diet (13.7% protein) for 5 days, followed by a high protein diet (HPD, 26.3% protein) for another 5 days. Urea cycle deficiency was monitored by the blood levels of citrulline and ammonia. HD progression was determined by the Unified Huntington's Disease Rating Scale (UHDRS). The HPD increased blood citrulline concentration from 15.19 μmol/l to 16.30 μmol/l (p = 0.0378) in HD patients but did not change blood ammonia concentration. A 2-year pilot study of 14 HD patients found no significant correlation between blood citrulline concentration and HD progression. Our results indicated a short period of the HPD did not markedly compromise urea cycle function. Blood citrulline concentration is not a reliable biomarker of HD progression.
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