Page last updated: 2024-10-19

thioctic acid and Ataxia with Lactic Acidosis

thioctic acid has been researched along with Ataxia with Lactic Acidosis in 5 studies

Thioctic Acid: An octanoic acid bridged with two sulfurs so that it is sometimes also called a pentanoic acid in some naming schemes. It is biosynthesized by cleavage of LINOLEIC ACID and is a coenzyme of oxoglutarate dehydrogenase (KETOGLUTARATE DEHYDROGENASE COMPLEX). It is used in DIETARY SUPPLEMENTS.

Research Excerpts

ExcerptRelevanceReference
"PDHc deficiency was demonstrated in muscle and fibroblasts without detectable PDHA1 mutations."1.36Dihydrolipoamide dehydrogenase (DLD) deficiency in a Spanish patient with myopathic presentation due to a new mutation in the interface domain. ( Briones, P; Font, A; Pineda, M; Quintana, E; Ribes, A; Tort, F; Vilaseca, MA, 2010)

Research

Studies (5)

TimeframeStudies, this research(%)All Research%
pre-19902 (40.00)18.7374
1990's1 (20.00)18.2507
2000's0 (0.00)29.6817
2010's2 (40.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Sperl, W1
Fleuren, L1
Freisinger, P1
Haack, TB1
Ribes, A2
Feichtinger, RG1
Rodenburg, RJ1
Zimmermann, FA1
Koch, J1
Rivera, I1
Prokisch, H1
Smeitink, JA1
Mayr, JA1
Quintana, E1
Pineda, M1
Font, A1
Vilaseca, MA1
Tort, F1
Briones, P1
Kodama, S1
Yagi, R1
Ninomiya, M1
Goji, K1
Takahashi, T1
Morishita, Y1
Matsuo, T1
Yoshida, I1
Sweetman, L1
Kulovich, S1
Nyhan, WL1
Robinson, BH1
Byrd, DJ1
Krohn, HP1
Winkler, L1
Steinborn, C1
Hadam, M1
Brodehl, J1
Hunneman, DH1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Phase 3 Trial of Dichloroacetate in Pyruvate Dehydrogenase Complex Deficiency:[NCT02616484]Phase 334 participants (Actual)Interventional2020-07-14Active, not recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

1 review available for thioctic acid and Ataxia with Lactic Acidosis

ArticleYear
The spectrum of pyruvate oxidation defects in the diagnosis of mitochondrial disorders.
    Journal of inherited metabolic disease, 2015, Volume: 38, Issue:3

    Topics: Energy Metabolism; Female; Humans; Iron-Sulfur Proteins; Male; Oxidation-Reduction; Pyruvate Dehydro

2015

Other Studies

4 other studies available for thioctic acid and Ataxia with Lactic Acidosis

ArticleYear
Dihydrolipoamide dehydrogenase (DLD) deficiency in a Spanish patient with myopathic presentation due to a new mutation in the interface domain.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: Acidosis, Lactic; Adult; Amino Acid Sequence; Base Sequence; Biomarkers; Blepharoptosis; Cells, Cult

2010
The effect of a high fat diet on pyruvate decarboxylase deficiency without central nervous system involvement.
    Brain & development, 1983, Volume: 5, Issue:4

    Topics: Carboxy-Lyases; Child; Dietary Fats; Electroencephalography; Fatigue; Fibroblasts; Humans; Male; Phy

1983
Effect of lipoic acid in a patient with defective activity of pyruvate dehydrogenase, 2-oxoglutarate dehydrogenase, and branched-chain keto acid dehydrogenase.
    Pediatric research, 1990, Volume: 27, Issue:1

    Topics: 3-Hydroxybutyric Acid; 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Acidosis, Lactic; Acidosis

1990
Neonatal pyruvate dehydrogenase deficiency with lipoate responsive lactic acidaemia and hyperammonaemia.
    European journal of pediatrics, 1989, Volume: 148, Issue:6

    Topics: Acidosis, Lactic; Ammonia; Female; Humans; Infant, Newborn; Pyruvate Dehydrogenase Complex Deficienc

1989