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pyruvic acid and Complex IV Deficiency

pyruvic acid has been researched along with Complex IV Deficiency in 18 studies

Pyruvic Acid: An intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
pyruvic acid : A 2-oxo monocarboxylic acid that is the 2-keto derivative of propionic acid. It is a metabolite obtained during glycolysis.

Research Excerpts

ExcerptRelevanceReference
" Patients with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes demonstrated only minor changes in redox state and in the behavior of the mitochondrial respiratory chain."3.68The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia. ( Capaldi, R; Chow, W; Glerum, DM; Lightowlers, R; Petrova-Benedict, R; Robinson, BH, 1990)
"Focal COX deficiency was found in 14 (93%) of 15 patients and in nine (60%) of 15 controls."1.31Impaired redox status and cytochrome c oxidase deficiency in patients with polymyalgia rheumatica. ( Authier, FJ; Chariot, P; Chevalier, X; Drogou, I; Gherardi, R; Yerroum, M, 2001)
"Here, we report on cytochrome c oxidase deficiency in the liver but not in the skeletal muscle of a 5-month-old girl who presented hepatic failure in early infancy."1.29Liver cytochrome c oxidase deficiency in a case of neonatal-onset hepatic failure. ( Cerrone, R; Chretien, D; Edery, P; Fabre, M; Gérard, B; Munnich, A; Rabier, D; Rambaud, C; Rötig, A; Saudubray, JM, 1994)
"To elucidate the etiology of Leigh syndrome, biochemical analyses and mitochondrial DNA analyses were performed on cultured lymphoblastoid cells from 20 patients with the clinical characteristics of this disorder."1.29[Defects of pyruvate metabolism in cultured lymphoblastoid cells of 20 patients with Leigh syndrome]. ( Ito, M; Kimura, S; Kuroda, Y; Matsuda, J; Naito, E; Osaka, H; Saijo, T; Yokota, I, 1996)
"Amniocytes with an established cytochrome c oxidase deficiency were also investigated."1.28Prenatal diagnosis of systemic disorders of the respiratory chain in cultured amniocytes and chorionic villus fibroblasts by studying the formation of lactate and pyruvate from glucose. ( Bakkeren, JA; Feller, N; Ruitenbeek, W; Ruiter, J; Sengers, RC; Trijbels, JM; Wanders, RJ; Wijburg, FA, 1992)
"The presentation and clinical course of cytochrome c oxidase deficiency are highly variable and the diagnosis of cytochrome c oxidase deficiency should be considered in all patients with lactic acidosis or subacute necrotizing encephalomyelopathy."1.28Variable presentation of cytochrome c oxidase deficiency. ( Cunniff, C; Keppler, K, 1992)
"Two new patients with Leigh's syndrome (subacute necrotizing encephalomyelopathy) due to deficiency of cytochrome c oxidase are presented and their data are compared with those of the four Leigh's syndrome patients previously reported with this deficiency."1.27Cytochrome c oxidase deficiency in subacute necrotizing encephalomyelopathy. ( Arts, WF; Fernandes, J; Loonen, MC; Luyt-Houwen, IE; Przyrembel, H; Scholte, HR; Trijbels, JM, 1987)

Research

Studies (18)

TimeframeStudies, this research(%)All Research%
pre-19904 (22.22)18.7374
1990's12 (66.67)18.2507
2000's2 (11.11)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Prick, MJ1
Gabreëls, FJ2
Trijbels, JM4
Janssen, AJ1
le Coultre, R1
van Dam, K1
Jaspar, HH1
Ebels, EJ1
Op de Coul, AA1
Edery, P1
Gérard, B1
Chretien, D1
Rötig, A1
Cerrone, R1
Rabier, D1
Rambaud, C1
Fabre, M1
Saudubray, JM1
Munnich, A1
Caruso, U1
Adami, A1
Bertini, E1
Burlina, AB1
Carnevale, F1
Cerone, R1
Dionisi-Vici, C1
Giordano, G1
Leuzzi, E1
Parenti, G1
Savasta, S1
Uziel, G1
Zeviani, M2
Bakker, HD1
Van den Bogert, C1
Drewes, JG1
Barth, PG1
Scholte, HR4
Wanders, RJ4
Ruitenbeek, W4
Naito, E1
Ito, M1
Yokota, I1
Saijo, T1
Matsuda, J1
Osaka, H1
Kimura, S1
Kuroda, Y1
Rubio-Gozalbo, ME1
Smeitink, JA1
Ter Laak, H1
Mullaart, RA1
Schuelke, M1
Mariman, EC1
Sengers, RC3
Murakami, N1
Sakuta, R1
Chariot, P1
Chevalier, X1
Yerroum, M1
Drogou, I1
Authier, FJ1
Gherardi, R1
Wijburg, FA3
Ruiter, J1
Bakkeren, JA1
Feller, N3
Keppler, K1
Cunniff, C1
Beyenburg, S1
von Wersebe, O1
Zierz, S1
Trounce, I1
Byrne, E1
Marzuki, S1
Dennett, X1
Sudoyo, H1
Mastaglia, F1
Berkovic, SF1
Sunohara, N1
Przyrembel, H3
Robinson, BH1
Glerum, DM1
Chow, W1
Petrova-Benedict, R1
Lightowlers, R1
Capaldi, R1
DiMauro, S1
Bonilla, E1
Servidei, S1
DeVivo, DC1
Schon, EA1
Arts, WF1
Loonen, MC1
Fernandes, J1
Luyt-Houwen, IE1

Reviews

3 reviews available for pyruvic acid and Complex IV Deficiency

ArticleYear
Respiratory-chain and pyruvate metabolism defects: Italian collaborative survey on 72 patients.
    Journal of inherited metabolic disease, 1996, Volume: 19, Issue:2

    Topics: Cytochrome-c Oxidase Deficiency; Electron Transport; Humans; Italy; Metabolism, Inborn Errors; Pyruv

1996
[Pure mitochondrial myopathy].
    Ryoikibetsu shokogun shirizu, 2001, Issue:36

    Topics: Child; Cytochrome-c Oxidase Deficiency; Electron Transport Complex I; Electron Transport Complex II;

2001
Mitochondrial myopathies.
    Journal of inherited metabolic disease, 1987, Volume: 10 Suppl 1

    Topics: Adenosine Triphosphatases; Biological Transport; Carnitine; Citric Acid Cycle; Cytochrome-c Oxidase

1987

Other Studies

15 other studies available for pyruvic acid and Complex IV Deficiency

ArticleYear
Progressive poliodystrophy (Alpers' disease) with a defect in cytochrome aa3 in muscle: a report of two unrelated patients.
    Clinical neurology and neurosurgery, 1983, Volume: 85, Issue:1

    Topics: Atrophy; Brain; Child; Child, Preschool; Cytochrome a Group; Cytochrome-c Oxidase Deficiency; Cytoch

1983
Liver cytochrome c oxidase deficiency in a case of neonatal-onset hepatic failure.
    European journal of pediatrics, 1994, Volume: 153, Issue:3

    Topics: Cytochrome-c Oxidase Deficiency; Female; Humans; Infant; Lactates; Lactic Acid; Liver; Liver Failure

1994
Progressive generalized brain atrophy and infantile spasms associated with cytochrome c oxidase deficiency.
    Journal of inherited metabolic disease, 1996, Volume: 19, Issue:2

    Topics: Acidosis; Atrophy; Brain; Cytochrome-c Oxidase Deficiency; Humans; Infant, Newborn; Lactates; Lactic

1996
[Defects of pyruvate metabolism in cultured lymphoblastoid cells of 20 patients with Leigh syndrome].
    No to hattatsu = Brain and development, 1996, Volume: 28, Issue:6

    Topics: Biomarkers; Cells, Cultured; Cytochrome-c Oxidase Deficiency; DNA, Mitochondrial; Female; Humans; In

1996
Spinal muscular atrophy-like picture, cardiomyopathy, and cytochrome c oxidase deficiency.
    Neurology, 1999, Jan-15, Volume: 52, Issue:2

    Topics: Blotting, Western; Cardiomegaly; Cells, Cultured; Cytochrome-c Oxidase Deficiency; Fibroblasts; Huma

1999
Impaired redox status and cytochrome c oxidase deficiency in patients with polymyalgia rheumatica.
    Annals of the rheumatic diseases, 2001, Volume: 60, Issue:11

    Topics: Aged; Aged, 80 and over; Biopsy; Case-Control Studies; Cytochrome-c Oxidase Deficiency; Female; Huma

2001
Prenatal diagnosis of systemic disorders of the respiratory chain in cultured amniocytes and chorionic villus fibroblasts by studying the formation of lactate and pyruvate from glucose.
    Journal of inherited metabolic disease, 1992, Volume: 15, Issue:1

    Topics: Amnion; Cells, Cultured; Chorionic Villi; Cytochrome-c Oxidase Deficiency; Female; Fibroblasts; Gluc

1992
Variable presentation of cytochrome c oxidase deficiency.
    American journal of diseases of children (1960), 1992, Volume: 146, Issue:11

    Topics: Cytochrome-c Oxidase Deficiency; Female; Humans; Infant, Newborn; Lactates; Lactic Acid; Male; Metab

1992
[Delayed manifestation of mitochondrial myopathy--complex I and IV deficiency of the mitochondrial respiratory chain with progressive paresis].
    Der Nervenarzt, 1991, Volume: 62, Issue:8

    Topics: Biopsy; Cytochrome-c Oxidase Deficiency; Electromyography; Humans; Lactates; Lactic Acid; Male; Midd

1991
Functional respiratory chain studies in subjects with chronic progressive external ophthalmoplegia and large heteroplasmic mitochondrial DNA deletions.
    Journal of the neurological sciences, 1991, Volume: 102, Issue:1

    Topics: Adult; Chromosome Deletion; Cytochrome-c Oxidase Deficiency; DNA, Mitochondrial; Electron Transport;

1991
[Leigh's syndrome].
    Nihon rinsho. Japanese journal of clinical medicine, 1990, Volume: 48, Issue:7

    Topics: Brain Diseases, Metabolic; Cytochrome-c Oxidase Deficiency; Electron Transport; Electron Transport C

1990
Detection of respiratory chain dysfunction by measuring lactate and pyruvate production in cultured fibroblasts.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Cells, Cultured; Child, Preschool; Cytochrome-c Oxidase Defici

1990
The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia.
    Pediatric research, 1990, Volume: 28, Issue:5

    Topics: Acidosis, Lactic; Brain Diseases; Cells, Cultured; Cytochrome-c Oxidase Deficiency; Fibroblasts; Hum

1990
Studies on the formation of lactate and pyruvate from glucose in cultured skin fibroblasts: implications for detection of respiratory chain defects.
    Biochemistry international, 1989, Volume: 19, Issue:3

    Topics: Cells, Cultured; Child, Preschool; Cytochrome-c Oxidase Deficiency; Electron Transport; Electron Tra

1989
Cytochrome c oxidase deficiency in subacute necrotizing encephalomyelopathy.
    Journal of the neurological sciences, 1987, Volume: 77, Issue:1

    Topics: Brain; Brain Diseases, Metabolic; Child; Child, Preschool; Cytochrome-c Oxidase Deficiency; Electron

1987