Page last updated: 2024-10-17

3-hydroxybutyric acid and Adult Sandhoff Disease

3-hydroxybutyric acid has been researched along with Adult Sandhoff Disease in 2 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.

Research Excerpts

ExcerptRelevanceReference
" These data suggest that the KD-R and NB-DNJ may be a potential combinatorial therapy for Sandhoff disease by enhancing NB-DNJ delivery to the brain and may allow lower dosing to achieve the same degree of efficacy as high dose monotherapy."1.36Restricted ketogenic diet enhances the therapeutic action of N-butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice. ( Baek, RC; Bronson, RT; Butters, TD; Denny, CA; Heinecke, KA; Kim, YP; Loh, KS; Platt, FM; Seyfried, TN, 2010)

Research

Studies (2)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (50.00)29.6817
2010's1 (50.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Denny, CA2
Heinecke, KA1
Kim, YP1
Baek, RC1
Loh, KS1
Butters, TD1
Bronson, RT2
Platt, FM1
Seyfried, TN2
Kasperzyk, JL1
Gorham, KN1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Synergistic Enteral Regimen for Treatment of the Gangliosidoses (Syner-G)[NCT02030015]Phase 416 participants (Actual)Interventional2015-12-22Terminated (stopped due to Efficacy)
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Other Studies

2 other studies available for 3-hydroxybutyric acid and Adult Sandhoff Disease

ArticleYear
Restricted ketogenic diet enhances the therapeutic action of N-butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice.
    Journal of neurochemistry, 2010, Volume: 113, Issue:6

    Topics: 1-Deoxynojirimycin; 3-Hydroxybutyric Acid; Analysis of Variance; Animals; beta-N-Acetylhexosaminidas

2010
Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice.
    Journal of neuroscience research, 2006, May-01, Volume: 83, Issue:6

    Topics: 3-Hydroxybutyric Acid; Age Factors; Animals; Antigens, CD; Antigens, Differentiation; Antigens, Diff

2006