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succinic acid and Electron Transport Chain Deficiencies, Mitochondrial

succinic acid has been researched along with Electron Transport Chain Deficiencies, Mitochondrial in 12 studies

Succinic Acid: A water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851)
succinic acid : An alpha,omega-dicarboxylic acid resulting from the formal oxidation of each of the terminal methyl groups of butane to the corresponding carboxy group. It is an intermediate metabolite in the citric acid cycle.

Research Excerpts

ExcerptRelevanceReference
"Liver is a target organ in many mitochondrial disorders, especially if the complex III assembly factor BCS1L is mutated."1.38Metabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex III deficiency due to a BCS1L mutation. ( Davoudi, M; Enot, DP; Fellman, V; Karikoski, R; Keller, M; Kotarsky, H; Levéen, P, 2012)
"Diagnosis of mitochondrial disorders usually requires a muscle biopsy to examine mitochondrial function."1.33Measurement of the energy-generating capacity of human muscle mitochondria: diagnostic procedure and application to human pathology. ( Janssen, AJ; Morava, E; Rodenburg, RJ; Ruitenbeek, W; Sengers, RC; Smeitink, JA; Trijbels, FJ; van den Heuvel, LP; van Engelen, BG; Wintjes, LT, 2006)

Research

Studies (12)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's7 (58.33)29.6817
2010's5 (41.67)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Miller, DM1
Singh, IN1
Wang, JA1
Hall, ED1
Roe, CR1
Brunengraber, H1
Ehinger, JK1
Piel, S1
Ford, R1
Karlsson, M1
Sjövall, F1
Frostner, EÅ1
Morota, S1
Taylor, RW1
Turnbull, DM1
Cornell, C1
Moss, SJ1
Metzsch, C1
Hansson, MJ1
Fliri, H1
Elmér, E1
Thaveau, F1
Zoll, J1
Bouitbir, J1
Ribera, F1
Di Marco, P1
Chakfe, N1
Kretz, JG1
Piquard, F1
Geny, B1
Orsucci, D1
Filosto, M1
Siciliano, G1
Mancuso, M1
Schiff, M1
Bénit, P2
El-Khoury, R1
Schlemmer, D1
Benoist, JF1
Rustin, P3
Kotarsky, H1
Keller, M1
Davoudi, M1
Levéen, P1
Karikoski, R1
Enot, DP1
Fellman, V1
Brière, JJ1
Chrétien, D1
Janssen, AJ1
Trijbels, FJ1
Sengers, RC1
Wintjes, LT1
Ruitenbeek, W1
Smeitink, JA1
Morava, E1
van Engelen, BG1
van den Heuvel, LP1
Rodenburg, RJ1
Vengerovskii, AI1
Khazanov, VA1
Eskina, KA1
Vasilyev, KY1
Rötig, A1
Kuroda, Y1
Naito, E1
Touda, Y1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
A Phase 2A/2B Placebo-controlled Randomised Clinical Trial to Test the Ability of Triheptanoin to Protect Primary Airway Epithelial Cells Obtained From Participants With Ataxia-telangiectasia Against Death Induced by Glucose Deprivation[NCT04513002]Phase 230 participants (Actual)Interventional2022-03-15Completed
Mitochondrial Targeted Biofuels as Countermeasures Against Chemical Threats[NCT03597438]700 participants (Anticipated)Observational2018-05-07Active, not recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

4 reviews available for succinic acid and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
Electron transfer mediators and other metabolites and cofactors in the treatment of mitochondrial dysfunction.
    Nutrition reviews, 2009, Volume: 67, Issue:8

    Topics: Acidosis, Lactic; Animals; Antioxidants; Carnitine; Creatine; Dietary Supplements; Electron Transpor

2009
Respiratory chain defects: what do we know for sure about their consequences in vivo?
    Biochimica et biophysica acta, 2004, Dec-06, Volume: 1659, Issue:2-3

    Topics: Adenosine Triphosphate; Cell Respiration; Humans; Mitochondria; Mitochondrial Diseases; Signal Trans

2004
Inborn errors of complex II--unusual human mitochondrial diseases.
    Biochimica et biophysica acta, 2002, Jan-17, Volume: 1553, Issue:1-2

    Topics: Citric Acid Cycle; Electron Transport Complex II; Flavoproteins; Fumarates; Humans; Metabolism, Inbo

2002
[Drug therapy for mitochondrial diseases].
    Nihon rinsho. Japanese journal of clinical medicine, 2002, Volume: 60 Suppl 4

    Topics: Carnitine; Coenzymes; Cytochrome c Group; Cytochromes c; Dichloroacetic Acid; Drug Combinations; Dru

2002

Other Studies

8 other studies available for succinic acid and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
Nrf2-ARE activator carnosic acid decreases mitochondrial dysfunction, oxidative damage and neuronal cytoskeletal degradation following traumatic brain injury in mice.
    Experimental neurology, 2015, Volume: 264

    Topics: Abietanes; Adenosine Diphosphate; Aldehydes; Analysis of Variance; Animals; Antioxidants; Brain; Bra

2015
Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience.
    Molecular genetics and metabolism, 2015, Volume: 116, Issue:4

    Topics: Administration, Oral; Adolescent; Adult; Carnitine; Child; Child, Preschool; Citric Acid; Fatty Acid

2015
Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency.
    Nature communications, 2016, 08-09, Volume: 7

    Topics: Cell Membrane Permeability; Cell Respiration; Drug Discovery; Drug Evaluation, Preclinical; Electron

2016
Contralateral leg as a control during skeletal muscle ischemia-reperfusion.
    The Journal of surgical research, 2009, Volume: 155, Issue:1

    Topics: Animals; Ascorbic Acid; Cell Respiration; Electron Transport Chain Complex Proteins; Glutamic Acid;

2009
Mouse studies to shape clinical trials for mitochondrial diseases: high fat diet in Harlequin mice.
    PloS one, 2011, Volume: 6, Issue:12

    Topics: Animals; Atrophy; Carnitine; Cattle; Cell Death; Cell Respiration; Cerebellum; Clinical Trials as To

2011
Metabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex III deficiency due to a BCS1L mutation.
    PloS one, 2012, Volume: 7, Issue:7

    Topics: Adenosine Monophosphate; Animals; Antioxidants; ATPases Associated with Diverse Cellular Activities;

2012
Measurement of the energy-generating capacity of human muscle mitochondria: diagnostic procedure and application to human pathology.
    Clinical chemistry, 2006, Volume: 52, Issue:5

    Topics: Adenosine Triphosphate; Adolescent; Adult; Biopsy; Carbon Radioisotopes; Carnitine; Child; Child, Pr

2006
Effects of silymarin (hepatoprotector) and succinic acid (bioenergy regulator) on metabolic disorders in experimental diabetes mellitus.
    Bulletin of experimental biology and medicine, 2007, Volume: 144, Issue:1

    Topics: Animals; Blood Glucose; Cholesterol; Diabetes Mellitus, Experimental; Lipid Peroxidation; Male; Mice

2007