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valerates and Metabolism, Inborn Errors

valerates has been researched along with Metabolism, Inborn Errors in 19 studies

Research

Studies (19)

TimeframeStudies, this research(%)All Research%
pre-199013 (68.42)18.7374
1990's4 (21.05)18.2507
2000's2 (10.53)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Baumgartner, MR; Baykal, T; Can, G; Dantas, MF; Demir, F; Demirkol, M; Fowler, B; Gokcay, GH; Ince, Z1
Bumack, C; Burke, D; Danks, DM; Haan, EA; Halpern, B; Hammond, J; Hick, L; Malegan, D; McCairns, E; Nyhan, WL; Sims, P; Sweetman, L; Tanaka, K; Truscott, RJ1
Duran, M; Kamerling, JP; Wadman, SK1
de Jong, JG; Engelke, U; Gabreëls, FJ; Heerschap, A; Wendel, U; Wevers, RA1
Berry, SA; Nyhan, WL; Thuy, LP; Tuchman, M1
Baumgartner, ER; Lehnert, W; Niederhoff, H; Suormala, T1
Bergh, JJ; Castagnoli, N; Mienie, LJ; Petzer, JP; Van der Schyf, CJ1
Danks, DM; Haan, E; Halpern, B; Hammond, J; Pullin, CJ; Truscott, RJ1
Nakamura, E; Rosenberg, LE; Tanaka, K1
Buchanan, DN; Muenzer, J; Thoene, JG1
Dinesen, J; Gerdes, AM; Gregersen, N; Güttler, F; Holm, V; Lúdvigsson, P1
Hyman, DB; Ikeda, Y; Matsubara, Y; Tanaka, K1
Callaghan, P; Newman, CG; Wilson, BD; Young, L1
Eldjarn, L; Jellum, E; Stokke, O1
Gompertz, D1
Ando, T; Bachmann, C; Nyhan, WL; Rasmussen, K; Scott, R; Smith, EK1
Barness, LA1
Goedde, HW; Langenbeck, U; Rüdiger, HW; Schulze-Schencking, M1
Goodman, SI; Hammond, KB1

Reviews

2 review(s) available for valerates and Metabolism, Inborn Errors

ArticleYear
Molecular basis of isovaleric acidemia and medium-chain acyl-CoA dehydrogenase deficiency.
    Enzyme, 1987, Volume: 38, Issue:1-4

    Topics: Acyl-CoA Dehydrogenases; Amino Acid Sequence; Base Sequence; DNA, Circular; Hemiterpenes; Humans; Isovaleryl-CoA Dehydrogenase; Metabolism, Inborn Errors; Molecular Sequence Data; Mutation; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pentanoic Acids; Protein Biosynthesis; Sequence Homology, Nucleic Acid; Valerates

1987
Inborn errors of organic acid metabolism.
    Clinics in endocrinology and metabolism, 1974, Volume: 3, Issue:1

    Topics: Adult; Amino Acid Metabolism, Inborn Errors; Biotin; Carboxylic Acids; Chromatography, Gas; Coenzyme A; Crotonates; Female; Gestational Age; Humans; Hydroxy Acids; Hydroxybutyrates; Infant; Infant, Newborn; Isomerases; Leucine; Male; Malonates; Metabolism, Inborn Errors; Pedigree; Pregnancy; Prenatal Diagnosis; Propionates; Pyrrolidonecarboxylic Acid; Valerates; Vitamin B 12

1974

Other Studies

17 other study(ies) available for valerates and Metabolism, Inborn Errors

ArticleYear
Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:2

    Topics: Carbon-Carbon Ligases; Consanguinity; Diagnosis, Differential; Fatal Outcome; Glycine; Humans; Infant; Infant, Newborn; Leukoencephalitis, Acute Hemorrhagic; Male; Metabolism, Inborn Errors; Mutation; RNA Splice Sites; Valerates

2005
New metabolites in isovaleric acidemia.
    Clinica chimica acta; international journal of clinical chemistry, 1981, Mar-05, Volume: 110, Issue:2-3

    Topics: 4-Butyrolactone; Chemical Phenomena; Chemistry; Gas Chromatography-Mass Spectrometry; Hemiterpenes; Humans; Infant, Newborn; Male; Maleates; Metabolism, Inborn Errors; Pentanoic Acids; Valerates

1981
Organic acidurias: approach, results and clinical relevance.
    Ciba Foundation symposium, 1982, Volume: 87

    Topics: Child; Child, Preschool; Crotonates; Female; Gas Chromatography-Mass Spectrometry; Glutarates; Humans; Infant; Keto Acids; Leucine; Ligases; Male; Metabolism, Inborn Errors; Oxidoreductases; Oxo-Acid-Lyases; Pregnancy; Prenatal Diagnosis; Valerates

1982
Standardized method for high-resolution 1H-NMR of cerebrospinal fluid.
    Clinical chemistry, 1995, Volume: 41, Issue:5

    Topics: Adult; Amino Acids; Canavan Disease; Carbon-Carbon Ligases; Cerebrospinal Fluid; Child; Female; Gas Chromatography-Mass Spectrometry; Histidine; Humans; Hydrogen-Ion Concentration; Lactates; Lactic Acid; Ligases; Magnetic Resonance Spectroscopy; Male; Metabolism, Inborn Errors; Sensitivity and Specificity; Valerates; Volatilization

1995
Partial methylcrotonyl-coenzyme A carboxylase deficiency in an infant with failure to thrive, gastrointestinal dysfunction, and hypertonia.
    Pediatrics, 1993, Volume: 91, Issue:3

    Topics: Biotin; Carbon-Carbon Ligases; Failure to Thrive; Gastrointestinal Diseases; Glycine; Humans; Infant; Ligases; Male; Metabolism, Inborn Errors; Muscle Hypertonia; Valerates

1993
Isolated biotin-resistant 3-methylcrotonyl-CoA carboxylase deficiency: long-term outcome in a case with neonatal onset.
    European journal of pediatrics, 1996, Volume: 155, Issue:7

    Topics: Age of Onset; Biotin; Carbon-Carbon Ligases; Developmental Disabilities; Diet, Protein-Restricted; Epilepsy, Generalized; Humans; Infant, Newborn; Ligases; Male; Metabolism, Inborn Errors; Patient Compliance; Valerates

1996
Metabolic defects caused by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and by HPTP (the tetrahydropyridinyl analog of haloperidol), in rats.
    Life sciences, 2000, Apr-07, Volume: 66, Issue:20

    Topics: 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine; Animals; Antipsychotic Agents; Haloperidol; Lactic Acid; Male; Metabolism, Inborn Errors; Mitochondria; Oxygen Consumption; Rats; Rats, Sprague-Dawley; Valerates

2000
The identification of 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid in a patient with propionic acidemia.
    Biomedical mass spectrometry, 1979, Volume: 6, Issue:7

    Topics: Gas Chromatography-Mass Spectrometry; Humans; Hydroxy Acids; Infant, Newborn; Keto Acids; Ketones; Male; Mass Spectrometry; Metabolism, Inborn Errors; Pentanoic Acids; Propionates; Valerates

1979
Microdetermination of methylmalonic acid and other short chain dicarboxylic acids by gas chromatography: use in prenatal diagnosis of methylmalonic acidemia and in studies of isovaleric acidemia.
    Clinica chimica acta; international journal of clinical chemistry, 1976, Apr-15, Volume: 68, Issue:2

    Topics: Adult; Child; Chromatography, Gas; Colorimetry; Dicarboxylic Acids; Evaluation Studies as Topic; Female; Fetus; Heterozygote; Humans; Infant, Newborn; Malonates; Metabolism, Inborn Errors; Methylmalonic Acid; Microchemistry; Pregnancy; Prenatal Diagnosis; Succinates; Valerates

1976
Positive-ion thermospray liquid chromatography-mass spectrometry: detection of organic acidurias.
    Journal of chromatography, 1990, Dec-14, Volume: 534

    Topics: Argininosuccinic Acid; Carboxylic Acids; Chromatography, Thin Layer; Female; Humans; Infant; Infant, Newborn; Male; Mass Spectrometry; Meglutol; Metabolism, Inborn Errors; Methylmalonic Acid; Sensitivity and Specificity; Valerates

1990
[Isovaleric acidemia].
    Ugeskrift for laeger, 1989, Oct-23, Volume: 151, Issue:43

    Topics: Acidosis; Child, Preschool; Dietary Proteins; Female; Humans; Infant; Male; Metabolism, Inborn Errors; Oxidoreductases; Valerates

1989
Neonatal death associated with isovalericacidaemia.
    Lancet (London, England), 1967, Aug-26, Volume: 2, Issue:7513

    Topics: Acidosis; Bicarbonates; Chromatography; Chromatography, Gas; Coenzyme A; Electrolytes; Fatty Acids; Fatty Acids, Nonesterified; Glucose; Hemorrhage; Humans; Hypocalcemia; Infant Mortality; Infant, Newborn; Leucine; Male; Metabolism, Inborn Errors; Valerates

1967
Combined use of gas chromatography, mass spectrometry, and computer in diagnosis and studies of metabolic disorders.
    Clinical chemistry, 1972, Volume: 18, Issue:8

    Topics: Adult; Biopsy; Blood Chemical Analysis; Butyrates; Carbon Dioxide; Chemistry, Clinical; Chromatography, Gas; Computers; Diagnosis, Computer-Assisted; Female; Glycine; Humans; Infant; Infant, Newborn; Ligases; Male; Malonates; Mass Spectrometry; Metabolic Diseases; Metabolism, Inborn Errors; Methods; Pentanols; Pyrrolidonecarboxylic Acid; Valerates

1972
Isovaleric acidemia: identification of isovalerate, isovalerylglycine, and 3-hydroxyisovalerate in urine of a patient previously reported as having butyric and hexanoic acidemia.
    The Journal of pediatrics, 1973, Volume: 82, Issue:2

    Topics: Butyrates; Caproates; Child; Chromatography, Gas; Chromatography, Thin Layer; Coenzyme A; Glycine; Humans; Mass Spectrometry; Metabolism, Inborn Errors; Valerates

1973
Methylmalonic acid.
    Pediatrics, 1973, Volume: 51, Issue:6

    Topics: Acidosis; Awards and Prizes; Child; Coenzymes; Female; Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Isomerases; Lactates; Malonates; Metabolism, Inborn Errors; Methane; Pregnancy; Propionates; Pyruvates; Succinates; Valerates; Vitamin B 12; Vitamin B 12 Deficiency

1973
Defective decarboxylase in branched chain ketoacid oxidase multienzyme complex in classic type of maple syrup urine disease.
    Humangenetik, 1972, Volume: 14, Issue:4

    Topics: Carboxy-Lyases; Decarboxylation; Female; Humans; Infant, Newborn; Keto Acids; Kinetics; Lipids; Maple Syrup Urine Disease; Metabolism, Inborn Errors; Molecular Biology; Oxidoreductases; Tissue Extracts; Valerates

1972
A gas chromatographic procedure for detection of pathological organic aciduria.
    Clinical chemistry, 1970, Volume: 16, Issue:3

    Topics: Acetates; Acids; Butyrates; Caproates; Chromatography, Gas; Dicarboxylic Acids; Glycols; Humans; Infant, Newborn; Lactates; Malonates; Metabolism, Inborn Errors; Methods; Methylation; Propionates; Solvents; Succinates; Valerates

1970