Page last updated: 2024-10-17

3-hydroxybutyric acid and Huntington Disease

3-hydroxybutyric acid has been researched along with Huntington Disease in 4 studies

3-Hydroxybutyric Acid: BUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.
3-hydroxybutyric acid : A straight-chain 3-hydroxy monocarboxylic acid comprising a butyric acid core with a single hydroxy substituent in the 3- position; a ketone body whose levels are raised during ketosis, used as an energy source by the brain during fasting in humans. Also used to synthesise biodegradable plastics.

Huntington Disease: A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

Research Excerpts

ExcerptRelevanceReference
"Huntington's disease is a fatal neurodegenerative disease, where dysfunction and loss of striatal and cortical neurons are central to the pathogenesis of the disease."1.48Integrative Characterization of the R6/2 Mouse Model of Huntington's Disease Reveals Dysfunctional Astrocyte Metabolism. ( Aldana, BI; Andersen, JV; Nielsen, ML; Nørremølle, A; Santos, A; Skotte, NH; Waagepetersen, HS; Willert, CW, 2018)
"Progressive weight loss is a hallmark feature of Huntington's disease, yet we found that the ketogenic diet-which generally causes weight loss in normal animals-delayed the reduction in body weight of the transgenic mice."1.37A ketogenic diet delays weight loss and does not impair working memory or motor function in the R6/2 1J mouse model of Huntington's disease. ( Geiger, JD; Kawamura, M; Masino, SA; Ross, JL; Ruiz, TL; Ruskin, DN, 2011)

Research

Studies (4)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's4 (100.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Skotte, NH1
Andersen, JV1
Santos, A1
Aldana, BI1
Willert, CW1
Nørremølle, A1
Waagepetersen, HS1
Nielsen, ML1
Chen, JY1
Tran, C1
Hwang, L1
Deng, G1
Jung, ME1
Faull, KF1
Levine, MS1
Cepeda, C1
Ruskin, DN1
Ross, JL1
Kawamura, M1
Ruiz, TL1
Geiger, JD1
Masino, SA1
Lim, S1
Chesser, AS1
Grima, JC1
Rappold, PM1
Blum, D1
Przedborski, S1
Tieu, K1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Keto-diet for Intubated Critical Care COVID-19 (KICC-COVID19)[NCT04358835]0 participants (Actual)Interventional2020-09-01Withdrawn (stopped due to Study did not begin enrollment, multiple competing studies at same institution)
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Other Studies

4 other studies available for 3-hydroxybutyric acid and Huntington Disease

ArticleYear
Integrative Characterization of the R6/2 Mouse Model of Huntington's Disease Reveals Dysfunctional Astrocyte Metabolism.
    Cell reports, 2018, 05-15, Volume: 23, Issue:7

    Topics: 3-Hydroxybutyric Acid; Acetates; Animals; Astrocytes; Brain; Disease Models, Animal; Energy Metaboli

2018
Partial Amelioration of Peripheral and Central Symptoms of Huntington's Disease via Modulation of Lipid Metabolism.
    Journal of Huntington's disease, 2016, Volume: 5, Issue:1

    Topics: 3-Hydroxybutyric Acid; Animals; Cholesterol; Diet, Ketogenic; Female; Huntington Disease; Inhibitory

2016
A ketogenic diet delays weight loss and does not impair working memory or motor function in the R6/2 1J mouse model of Huntington's disease.
    Physiology & behavior, 2011, Jul-06, Volume: 103, Issue:5

    Topics: 3-Hydroxybutyric Acid; Age Factors; Animals; Diet, Ketogenic; Disease Models, Animal; Female; Humans

2011
D-β-hydroxybutyrate is protective in mouse models of Huntington's disease.
    PloS one, 2011, Volume: 6, Issue:9

    Topics: 3-Hydroxybutyric Acid; Acetylation; Animals; Histones; Huntington Disease; Immunoblotting; Male; Mic

2011