valerates has been researched along with BCKD Deficiency in 19 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 17 (89.47) | 18.7374 |
| 1990's | 1 (5.26) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 1 (5.26) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Amaral, AU; Fernandes, CG; Leipnitz, G; Schuck, PF; Seminotti, B; Wajner, M | 1 |
| Jakobs, C; Nyhan, WL; Sweetman, L | 1 |
| Goldstein, F; Norton, PM; Sansaricq, C; Snyderman, SE | 1 |
| Burke, DG; Danks, D; Halpern, B; Malegan, D; McCairns, E; Schlesinger, P; Wilken, B | 1 |
| Ckless, K; Schlottfeldt, JL; Wajner, M; Wannmacher, CM | 1 |
| Goedde, HW; Singh, S; Willers, I | 1 |
| Piccardo, M; Tassara, F; Vico, L | 1 |
| Danner, DJ; Elsas, LJ; Lemmon, SK; Wheeler, FB | 1 |
| Mandell, R; Shih, VE; Tanaka, K | 1 |
| Hagenfeldt, L; Naglo, AS | 1 |
| Goedde, HW; Langenbeck, U; Passarge, E; Rüdiger, HW; Wendel, U; Wöhler, W | 1 |
| Lancaster, G; Mamer, OA; Scriver, CR | 1 |
| Angielski, SA; Lysiak, W; Pieńkowska-Vogel, M; Szutowicz, A | 1 |
| Bensch, KG; Bissell, MG; Herman, MM | 1 |
| Goedde, HW; Langenbeck, U; Rüdiger, HW; Schulze-Schencking, M | 1 |
| Carpenter, DG; Carter, CH | 1 |
| Angielski, S; Lysiak, W; Stepiński, J | 1 |
| Bowden, JA; Connelly, JL; Danner, DJ | 1 |
| Fukuyoshi, Y; Hamada, M; Hayakawa, T; Kanzaki, T; Koike, M | 1 |
1 review(s) available for valerates and BCKD Deficiency
| Article | Year |
|---|---|
[Some clinical and therapeutic aspects of organico-acidemia and organico-aciduria].
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Butyrates; Humans; Maple Syrup Urine Disease; Metabolic Diseases; Methylmalonic Acid; Propionates; Renal Aminoacidurias; Valerates | 1977 |
18 other study(ies) available for valerates and BCKD Deficiency
| Article | Year |
|---|---|
Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain.
Topics: Animals; Brain; Central Nervous System Agents; Electron Transport; Homeostasis; Keto Acids; Ketoglutarate Dehydrogenase Complex; Leucine; Maple Syrup Urine Disease; Membrane Potential, Mitochondrial; Mitochondrial Diseases; Mitochondrial Swelling; NADP; Oxygen Consumption; Prosencephalon; Rats; Rats, Wistar; Valerates | 2010 |
Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Amniotic Fluid; Caproates; Female; Gas Chromatography-Mass Spectrometry; Humans; Hydroxy Acids; Hydroxybutyrates; Maple Syrup Urine Disease; Methylmalonic Acid; Pentanoic Acids; Pregnancy; Prenatal Diagnosis; Valerates | 1984 |
The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease.
Topics: Amino Acids, Branched-Chain; Caproates; Child; Child, Preschool; Female; Hemiterpenes; Humans; Infant; Infant, Newborn; Keto Acids; Leucine; Male; Maple Syrup Urine Disease; Valerates; Valine | 1984 |
Profiles of urinary volatiles from metabolic disorders characterized by unusual odors.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Chromatography, Gas; Flame Ionization; Hemiterpenes; Humans; Infant, Newborn; Maple Syrup Urine Disease; Methylamines; Pentanoic Acids; Phenylketonurias; Valerates | 1983 |
Immunosuppressive effects of organic acids accumulating in patients with maple syrup urine disease.
Topics: Caproates; Cells, Cultured; Hemiterpenes; Humans; Keto Acids; Lymphocyte Activation; Maple Syrup Urine Disease; Mitogens; Pentanoic Acids; Phytohemagglutinins; Valerates | 1995 |
Heterogeneity in maple syrup urine disease: aspects of cofactor requirement and complementation in cultured fibroblasts.
Topics: Caproates; Carboxy-Lyases; Cells, Cultured; Coenzyme A; Decarboxylation; Fibroblasts; Humans; Hybrid Cells; Keto Acids; Maple Syrup Urine Disease; NAD; Thiamine Pyrophosphate; Valerates | 1977 |
In vivo and in vitro response of human branched chain alpha-ketoacid dehydrogenase to thiamine and thiamine pyrophosphate.
Topics: Fibroblasts; Hot Temperature; Humans; In Vitro Techniques; Keto Acids; Ketone Oxidoreductases; Leukocytes; Male; Maple Syrup Urine Disease; Mitochondria; Thiamine; Thiamine Pyrophosphate; Valerates | 1978 |
Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects.
Topics: 2-Methyl-4-chlorophenoxyacetic Acid; Amino Acid Metabolism, Inborn Errors; Carbon Dioxide; Cell Line; Cells, Cultured; Depression, Chemical; Fibroblasts; Flavin-Adenine Dinucleotide; Glycine; Humans; Leucine; Maple Syrup Urine Disease; Oxidation-Reduction; Skin; Succinates; Valerates; Valine | 1976 |
New conjugated urinary metabolites in intermediate type maple syrup urine disease.
Topics: Amino Acids; Amino Acids, Branched-Chain; Carboxylic Acids; Female; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant; Lactates; Lactic Acid; Maple Syrup Urine Disease; Valerates | 1987 |
Rapid diagnosis of maple syrup urine disease (branched chain ketoaciduria) by micro-enzyme assay in leukocytes and fibroblasts.
Topics: Caproates; Carbon Isotopes; Carboxy-Lyases; Cells, Cultured; Clinical Enzyme Tests; Fibroblasts; Heterozygote; Homozygote; Humans; Kinetics; Leukocytes; Maple Syrup Urine Disease; Methods; Microchemistry; Skin; Time Factors; Valerates | 1973 |
Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.
Topics: Amino Acids; Caproates; Chromatography, Gas; Diet; Fatty Acids; Humans; Keto Acids; Maple Syrup Urine Disease; Oximes; Pentanols; Phenotype; Pyruvate Dehydrogenase Complex; Thiamine; Valerates | 1974 |
Inhibition of alanine and aspartate aminotransferases by alpha-oxoderivatives of the branched-chain amino acids.
Topics: Alanine Transaminase; Amino Acids; Animals; Aspartate Aminotransferases; Azoles; Binding Sites; Brain; Caproates; Humans; Keto Acids; Kinetics; Leucine; Liver; Maple Syrup Urine Disease; Organ Specificity; Protein Binding; Pyridoxamine; Rats; Spectrophotometry, Ultraviolet; Transaminases; Valerates | 1974 |
Effects of maple syrup urine disease metabolites on mouse L-fibroblasts in vitro: a fine structural and biochemical study.
Topics: Animals; Caprylates; Carbon Radioisotopes; Cell Count; Cell Division; Chromatography, Thin Layer; Humans; Hydrogen-Ion Concentration; Isoleucine; Keto Acids; L Cells; Leucine; Maple Syrup Urine Disease; Mice; Microscopy, Electron; Time Factors; Tritium; Valerates | 1974 |
Defective decarboxylase in branched chain ketoacid oxidase multienzyme complex in classic type of maple syrup urine disease.
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 |
Inborn errors of metabolism associated with unusual odors.
Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Fatty Acids; Female; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Malabsorption Syndromes; Maple Syrup Urine Disease; Methionine; Odorants; Phenylalanine; Tryptophan; Valerates | 1970 |
Inhibition of alpha-oxoglutarate and pyruvate oxidation by alpha-oxoderivatives of leucine and valine in rat tissues.
Topics: Animals; Brain; Caproates; Citrates; Coenzyme A; Humans; Keto Acids; Ketoglutaric Acids; Kidney; Kinetics; Leucine; Malates; Maple Syrup Urine Disease; Metabolism; Mitochondria; Mitochondria, Liver; Mitochondria, Muscle; Myocardium; Oxidoreductases; Pyruvates; Rats; Valerates; Valine; Vibration | 1970 |
Branched chain alpha-keto acid metabolism. I. Isolation, purification, and partial characterization of bovine liver alpha-ketoisocaproic:alpha-keto-beta-methylvaleric acid dehydrogenase.
Topics: Animals; Caproates; Carbon Isotopes; Carboxy-Lyases; Cattle; Centrifugation; Chemical Precipitation; Guinea Pigs; Humans; Hydrogen-Ion Concentration; Keto Acids; Kinetics; Liver; Maple Syrup Urine Disease; Mice; Oxidoreductases; Rabbits; Rats; Swine; Valerates | 1968 |
Mammalian alpha-keto acid dehydrogenase complexes. IV. Substrate specificities and kinetic properties of the pig heart pyruvate and 2-oxyoglutarate dehydrogenase complexes.
Topics: Adipates; Animals; Butyrates; Caproates; Chemical Phenomena; Chemistry; Humans; Hydrogen-Ion Concentration; Keto Acids; Ketoglutaric Acids; Kinetics; Maple Syrup Urine Disease; Myocardium; Oxidoreductases; Oxygen Consumption; Pyruvates; Swine; Valerates | 1969 |