Page last updated: 2024-10-17

3-hydroxybutyric acid and Lipid Metabolism, Inborn Error

3-hydroxybutyric acid has been researched along with Lipid Metabolism, Inborn Error in 8 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
" Metabolic changes (liver steatosis and loss of muscle glycogen stores) appear also early after carnitine deprivation."3.74Effect of carnitine deprivation on carnitine homeostasis and energy metabolism in mice with systemic carnitine deficiency. ( Beier, K; Knapp, AC; Krähenbühl, S; Todesco, L; Torok, M, 2008)
"Triheptanoin is an odd-carbon, medium-chain triglyceride consisting of 3 fatty acids with 7 carbons each on a glycerol backbone developed to treat adult and pediatric patients with LC-FAODs."3.01The Pharmacokinetics of Triheptanoin and Its Metabolites in Healthy Subjects and Patients With Long-Chain Fatty Acid Oxidation Disorders. ( Gupta, M; Lee, SK; McKeever, K; Shi, J, 2021)

Research

Studies (8)

TimeframeStudies, this research(%)All Research%
pre-19902 (25.00)18.7374
1990's3 (37.50)18.2507
2000's1 (12.50)29.6817
2010's1 (12.50)24.3611
2020's1 (12.50)2.80

Authors

AuthorsStudies
Lee, SK1
Gupta, M1
Shi, J1
McKeever, K1
Semeraro, M1
Boenzi, S1
Carrozzo, R1
Diodato, D1
Martinelli, D1
Olivieri, G1
Antonetti, G1
Sacchetti, E1
Catesini, G1
Rizzo, C1
Dionisi-Vici, C1
Knapp, AC1
Todesco, L1
Torok, M1
Beier, K1
Krähenbühl, S1
Treacy, E1
Pitt, J1
Eggington, M1
Hawkins, R1
Wanders, RJ1
Ijlst, L1
Bonnefont, JP1
Specola, NB1
Vassault, A1
Lombes, A1
Ogier, H1
de Klerk, JB1
Munnich, A1
Coude, M1
Paturneau-Jouas, M1
Saudubray, JM1
Treem, WR1
Stanley, CA1
Goodman, SI1
Amendt, BA1
Greene, C1
Sweetman, L1
Cloherty, J1
Shih, V1
Moon, A1
Teel, L1
Rhead, WJ1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Fasting Tolerance in Patients With Medium-chain Acyl-CoA Dehydrogenase Deficiency (MCADD) in the First Six Months of Life: an Investigator-initiated Human Pilot-study[NCT03761693]20 participants (Anticipated)Interventional2019-05-15Recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trials

1 trial available for 3-hydroxybutyric acid and Lipid Metabolism, Inborn Error

ArticleYear
The Pharmacokinetics of Triheptanoin and Its Metabolites in Healthy Subjects and Patients With Long-Chain Fatty Acid Oxidation Disorders.
    Clinical pharmacology in drug development, 2021, Volume: 10, Issue:11

    Topics: 3-Hydroxybutyric Acid; Adolescent; Adult; Child; Cross-Over Studies; Fatty Acids; Female; Healthy Vo

2021

Other Studies

7 other studies available for 3-hydroxybutyric acid and Lipid Metabolism, Inborn Error

ArticleYear
The urinary organic acids profile in single large-scale mitochondrial DNA deletion disorders.
    Clinica chimica acta; international journal of clinical chemistry, 2018, Volume: 481

    Topics: 3-Hydroxybutyric Acid; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Child; Child, Preschool; Cong

2018
Effect of carnitine deprivation on carnitine homeostasis and energy metabolism in mice with systemic carnitine deficiency.
    Annals of nutrition & metabolism, 2008, Volume: 52, Issue:2

    Topics: 3-Hydroxybutyric Acid; Adenosine Triphosphate; Animals; Body Weight; Carnitine; Energy Metabolism; F

2008
Dicarboxylic aciduria, significance and prognostic indications.
    European journal of pediatrics, 1994, Volume: 153, Issue:12

    Topics: 3-Hydroxybutyric Acid; Acyl-CoA Dehydrogenase; Adipates; Child; Child, Preschool; Diagnosis, Differe

1994
Fatty acid beta-oxidation in leukocytes from control subjects and medium-chain acyl-CoA dehydrogenase deficient patients.
    Biochimica et biophysica acta, 1992, Jan-16, Volume: 1138, Issue:1

    Topics: 3-Hydroxybutyric Acid; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Caprylates; Fatty Acids; Hum

1992
The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states.
    European journal of pediatrics, 1990, Volume: 150, Issue:2

    Topics: 3-Hydroxybutyric Acid; Adolescent; Blood Glucose; Child; Child, Preschool; Fasting; Fatty Acids, Non

1990
Medium-chain acyl-CoA dehydrogenase deficiency: metabolic effects and therapeutic efficacy of long-term L-carnitine supplementation.
    Journal of inherited metabolic disease, 1989, Volume: 12, Issue:2

    Topics: 3-Hydroxybutyric Acid; Acyl-CoA Dehydrogenases; Carnitine; Chromatography, Gas; Fasting; Fatty Acids

1989
Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients.
    The Journal of clinical investigation, 1987, Volume: 79, Issue:5

    Topics: 3-Hydroxybutyric Acid; Acyl-CoA Dehydrogenases; Adipates; Adult; Butyrates; Butyric Acid; Female; Fi

1987