2-keto-4-methylvalerate has been researched along with BCKD Deficiency in 40 studies
alpha-ketoisocaproic acid: RN given refers to parent cpd
4-methyl-2-oxopentanoate : A 2-oxo monocarboxylic acid anion that is the conjugate base of 4-methyl-2-oxopentanoic acid.
4-methyl-2-oxopentanoic acid : A 2-oxo monocarboxylic acid that is pentanoic acid (valeric acid) substituted with a keto group at C-2 and a methyl group at C-4. A metabolite that has been found to accumulate in maple syrup urine disease.
| Excerpt | Relevance | Reference |
|---|---|---|
| " Considering that brain energy metabolism is possibly altered in MSUD, the objective of this study was to determine creatine kinase (CK) activity, a key enzyme of energy homeostasis, in C6 glioma cells exposed to BCKA." | 7.73 | Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease. ( Funchal, C; Gottfried, C; Jacques-Silva, MC; Pessoa-Pureur, R; Santos, AQ; Schuck, PF; Wajner, M, 2006) |
| "Increased concentrations of leucine and its respective ketoacid alpha-ketoisocaproate (KIC) in plasma and cerebrospinal fluid are related to acute and reversible encephalopathy in patients with maple syrup urine disease." | 7.72 | Disturbance of cultured rat neuronal network activity depends on concentration and ratio of leucine and alpha-ketoisocaproate: implication for acute encephalopathy of maple syrup urine disease. ( Görtz, P; Köller, H; Schwahn, B; Siebler, M; Wendel, U, 2003) |
| "Suitability of a recently proposed noninvasive L-[13C]leucine breath test for assessment of whole body leucine oxidation in maple syrup urine disease (MSUD) was examined." | 7.70 | Assessment of whole body L-leucine oxidation by noninvasive L-[1-13C]leucine breath tests: a reappraisal in patients with maple syrup urine disease, obligate heterozygotes, and healthy subjects. ( Bodner, A; Brösicke, H; Hammen, HW; Schadewaldt, P; Wendel, U, 1998) |
| " Considering that brain energy metabolism is possibly altered in MSUD, the objective of this study was to determine creatine kinase (CK) activity, a key enzyme of energy homeostasis, in C6 glioma cells exposed to BCKA." | 3.73 | Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease. ( Funchal, C; Gottfried, C; Jacques-Silva, MC; Pessoa-Pureur, R; Santos, AQ; Schuck, PF; Wajner, M, 2006) |
| "Increased concentrations of leucine and its respective ketoacid alpha-ketoisocaproate (KIC) in plasma and cerebrospinal fluid are related to acute and reversible encephalopathy in patients with maple syrup urine disease." | 3.72 | Disturbance of cultured rat neuronal network activity depends on concentration and ratio of leucine and alpha-ketoisocaproate: implication for acute encephalopathy of maple syrup urine disease. ( Görtz, P; Köller, H; Schwahn, B; Siebler, M; Wendel, U, 2003) |
| "Suitability of a recently proposed noninvasive L-[13C]leucine breath test for assessment of whole body leucine oxidation in maple syrup urine disease (MSUD) was examined." | 3.70 | Assessment of whole body L-leucine oxidation by noninvasive L-[1-13C]leucine breath tests: a reappraisal in patients with maple syrup urine disease, obligate heterozygotes, and healthy subjects. ( Bodner, A; Brösicke, H; Hammen, HW; Schadewaldt, P; Wendel, U, 1998) |
| "Leucine and alpha-ketoisocaproate (alpha-KIC) were perfused at increasing concentrations into rat brain hippocampus by microdialysis to mimic the conditions of maple syrup urine disease." | 3.69 | Effect of alpha-ketoisocaproate and leucine on the in vivo oxidation of glutamate and glutamine in the rat brain. ( Baab, PJ; Collins, RM; Huang, Y; Tildon, JT; Zielke, CL; Zielke, HR, 1997) |
| "Maple syrup urine disease (MSUD), an inborn error of metabolism in humans, is expressed as an inability to oxidatively decarboxylate the branched-chain alpha-keto acids derived from leucine, isoleucine and valine." | 3.66 | Myelin proteins: degradation in rat brain initiated by metabolites causative of maple syrup urine disease. ( Shapira, R; Tribble, D, 1983) |
| "Maple syrup urine disease is a metabolic disorder caused by mutations of the branched chain keto acid dehydrogenase complex, leading to accumulation of alpha-keto acids and their amino acid precursors in the brain." | 1.31 | Chloride-dependent inhibition of vesicular glutamate uptake by alpha-keto acids accumulated in maple syrup urine disease. ( Farage, M; Reis, M; Wolosker, H, 2000) |
| "Maple syrup urine disease is an inherited metabolic disorder characterized by tissue accumulation of branched-chain amino acids and their corresponding keto acids in the affected children." | 1.31 | Inhibition of glutamate uptake into synaptic vesicles of rat brain by the metabolites accumulating in maple syrup urine disease. ( Dutra-Filho, CS; Santos, CE; Souza, DO; Tasca, CI; Tavares, RG; Wajner, M, 2000) |
| " KIV elicited clonic convulsions in a dose-response manner, whereas KIC and KMV did not induce seizure-like behavior." | 1.31 | Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats. ( Coitinho, AS; de Bastiani, J; de Mello, CF; Fighera, MR; Lima, TT; Wajner, M, 2001) |
| "KIV elicited clonic convulsions in a dose-response manner, whereas KIC and KMV did not induce seizure-like behavior." | 1.31 | Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats. ( Coitinho, AS; de Bastiani, J; de Mello, CF; Fighera, MR; Lima, TT; Wajner, M, 2001) |
| "Maple syrup urine disease (MSUD) and phenylketonuria (PKU) are associated with accumulation of large neutral amino acids (LNAA) in blood and tissues and a decrease of other LNAA not directly related to the enzyme defects." | 1.31 | Large neutral amino acids auto exchange when infused by microdialysis into the rat brain: implication for maple syrup urine disease and phenylketonuria. ( Baab, PJ; Collins, RM; Zielke, CL; Zielke, HR, 2002) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 13 (32.50) | 18.7374 |
| 1990's | 5 (12.50) | 18.2507 |
| 2000's | 17 (42.50) | 29.6817 |
| 2010's | 3 (7.50) | 24.3611 |
| 2020's | 2 (5.00) | 2.80 |
| Authors | Studies |
|---|---|
| Rabelo, F | 1 |
| Lemos, IDS | 1 |
| Dal Toé, CP | 1 |
| Casagrande, DD | 1 |
| Freitas, MLS | 1 |
| Quadra, MR | 1 |
| Lima, IR | 1 |
| Generoso, JS | 1 |
| Michels, M | 2 |
| Silveira, PCL | 1 |
| Pizzol, FD | 1 |
| Streck, EL | 3 |
| Farias, HR | 1 |
| Gabriel, JR | 1 |
| Cecconi, ML | 1 |
| Lemos, IS | 1 |
| de Rezende, VL | 1 |
| Wessler, LB | 1 |
| Duarte, MB | 1 |
| Scaini, G | 2 |
| de Oliveira, J | 1 |
| Taschetto, L | 1 |
| Zapelini, HG | 1 |
| Ramos, ÂC | 1 |
| Strapazzon, G | 1 |
| Andrade, VM | 1 |
| Réus, GZ | 1 |
| Dal-Pizzol, F | 1 |
| Quevedo, J | 1 |
| Schuck, PF | 4 |
| Ferreira, GC | 1 |
| S Sonnet, D | 1 |
| N O'Leary, M | 1 |
| A Gutierrez, M | 1 |
| M Nguyen, S | 1 |
| Mateen, S | 1 |
| Hsu, Y | 1 |
| P Mitchell, K | 1 |
| J Lopez, A | 1 |
| Vockley, J | 1 |
| K Kennedy, B | 1 |
| Ramanathan, A | 1 |
| Zinnanti, WJ | 1 |
| Lazovic, J | 1 |
| Griffin, K | 1 |
| Skvorak, KJ | 1 |
| Paul, HS | 1 |
| Homanics, GE | 1 |
| Bewley, MC | 1 |
| Cheng, KC | 1 |
| Lanoue, KF | 1 |
| Flanagan, JM | 1 |
| Amaral, AU | 1 |
| Leipnitz, G | 1 |
| Fernandes, CG | 1 |
| Seminotti, B | 1 |
| Wajner, M | 14 |
| Funchal, C | 6 |
| de Lima Pelaez, P | 2 |
| Loureiro, SO | 1 |
| Vivian, L | 2 |
| Dall Bello Pessutto, F | 1 |
| de Almeida, LM | 2 |
| Tchernin Wofchuk, S | 1 |
| Pessoa Pureur, R | 1 |
| Görtz, P | 1 |
| Köller, H | 1 |
| Schwahn, B | 1 |
| Wendel, U | 8 |
| Siebler, M | 1 |
| GOEDDE, HW | 1 |
| RICHTER, E | 1 |
| STAHLMANN, C | 1 |
| SIXEL, B | 1 |
| PICCARDO, MG | 1 |
| LUZIETTI, L | 1 |
| CAPURSO, L | 1 |
| Sgaravatti, AM | 1 |
| Rosa, RB | 1 |
| Ribeiro, CA | 1 |
| Wannmacher, CM | 4 |
| Wyse, AT | 1 |
| Dutra-Filho, CS | 4 |
| de Castro Vasques, V | 1 |
| de Boer, MA | 1 |
| Diligenti, F | 1 |
| Brinco, F | 1 |
| Mallmann, F | 1 |
| Mello, CF | 1 |
| Gottfried, C | 3 |
| Pessoa-Pureur, R | 5 |
| Bridi, R | 1 |
| Braun, CA | 1 |
| Zorzi, GK | 1 |
| Lissi, EG | 1 |
| Zamoner, A | 2 |
| dos Santos, AQ | 2 |
| Moretto, MB | 1 |
| Rocha, JB | 1 |
| Jacques-Silva, MC | 2 |
| Santos, AQ | 1 |
| Wajner, A | 1 |
| Bürger, C | 1 |
| de Souza Wyse, AT | 1 |
| Tribble, D | 1 |
| Shapira, R | 1 |
| Langenbeck, U | 2 |
| Snyderman, SE | 1 |
| Goldstein, F | 1 |
| Sansaricq, C | 1 |
| Norton, PM | 1 |
| Lombeck, I | 1 |
| Bremer, HJ | 1 |
| Hutson, SM | 1 |
| Harper, AE | 1 |
| Gamm, G | 1 |
| Claussen, U | 1 |
| Schlottfeldt, JL | 1 |
| Ckless, K | 1 |
| Gouyon, JB | 1 |
| Semama, D | 1 |
| Prévot, A | 1 |
| Desgres, J | 1 |
| Zielke, HR | 2 |
| Huang, Y | 1 |
| Baab, PJ | 2 |
| Collins, RM | 2 |
| Zielke, CL | 2 |
| Tildon, JT | 1 |
| Schadewaldt, P | 4 |
| Bodner, A | 1 |
| Brösicke, H | 1 |
| Hammen, HW | 2 |
| Ott, AC | 1 |
| Jouvet, P | 1 |
| Rustin, P | 1 |
| Taylor, DL | 1 |
| Pocock, JM | 1 |
| Felderhoff-Mueser, U | 1 |
| Mazarakis, ND | 1 |
| Sarraf, C | 1 |
| Joashi, U | 1 |
| Kozma, M | 1 |
| Greenwood, K | 1 |
| Edwards, AD | 1 |
| Mehmet, H | 1 |
| Reis, M | 1 |
| Farage, M | 1 |
| Wolosker, H | 1 |
| Tavares, RG | 1 |
| Santos, CE | 1 |
| Tasca, CI | 1 |
| Souza, DO | 1 |
| Coitinho, AS | 1 |
| de Mello, CF | 1 |
| Lima, TT | 1 |
| de Bastiani, J | 1 |
| Fighera, MR | 1 |
| Oliveira Loureiro, S | 1 |
| de Freitas Miranda, R | 1 |
| Liao, CL | 1 |
| Herman, MM | 1 |
| Bensch, KG | 1 |
| Hummel, W | 1 |
| Trautvetter, U | 1 |
| Beck, K | 2 |
| Radeck, W | 1 |
| Staib, W | 1 |
| Fernhoff, PM | 1 |
| Lubitz, D | 1 |
| Danner, DJ | 1 |
| Dembure, PP | 1 |
| Schwartz, HP | 1 |
| Hillman, R | 1 |
| Bier, DM | 1 |
| Elsas, LJ | 1 |
1 trial available for 2-keto-4-methylvalerate and BCKD Deficiency
| Article | Year |
|---|---|
Thiamine response in maple syrup urine disease.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Amino Acids, Branched-Chain; Child; Child, Presch | 1985 |
39 other studies available for 2-keto-4-methylvalerate and BCKD Deficiency
| Article | Year |
|---|---|
Acute effects of intracerebroventricular administration of α-ketoisocaproic acid in young rats on inflammatory parameters.
Topics: Amino Acids, Branched-Chain; Animals; Keto Acids; Male; Maple Syrup Urine Disease; Oxidative Stress; | 2023 |
The metabolic effect of α-ketoisocaproic acid: in vivo and in vitro studies.
Topics: Amino Acids, Branched-Chain; Animals; Cell Line; Hippocampus; Keto Acids; Male; Maple Syrup Urine Di | 2021 |
Acute and long-term effects of intracerebroventricular administration of α-ketoisocaproic acid on oxidative stress parameters and cognitive and noncognitive behaviors.
Topics: Animals; Avoidance Learning; Behavior, Animal; Catalase; Cognition; Injections, Intraventricular; Ke | 2017 |
Metformin inhibits Branched Chain Amino Acid (BCAA) derived ketoacidosis and promotes metabolic homeostasis in MSUD.
Topics: Amino Acids, Branched-Chain; Animals; Chromatography, Liquid; Disease Models, Animal; Female; Fibrob | 2016 |
Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease.
Topics: Amino Acids; Animals; Behavior, Animal; Brain; Brain Edema; Brain Mapping; Dietary Proteins; Disease | 2009 |
Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain.
Topics: Animals; Brain; Central Nervous System Agents; Electron Transport; Homeostasis; Keto Acids; Ketoglut | 2010 |
alpha-Ketoisocaproic acid regulates phosphorylation of intermediate filaments in postnatal rat cortical slices through ionotropic glutamatergic receptors.
Topics: Animals; Animals, Newborn; Cerebral Cortex; Excitatory Amino Acid Agonists; Excitatory Amino Acid An | 2002 |
Disturbance of cultured rat neuronal network activity depends on concentration and ratio of leucine and alpha-ketoisocaproate: implication for acute encephalopathy of maple syrup urine disease.
Topics: Animals; Cells, Cultured; Culture Media; Dose-Response Relationship, Drug; Electrophysiology; Keto A | 2003 |
[HETEROZYGOTE TEST FOR MAPLE SYRUP URINE DISEASE. DETERMINATION OF THE ENZYMATIC ACTIVITY OF ALPHA-KETOISOCAPROIC ACID OXIDASE IN LEUKOCYTES].
Topics: Genetics, Medical; Heterozygote; Humans; Keto Acids; Leukocytes; Maple Syrup Urine Disease; Oxidored | 1963 |
[VARIOUS ASPECTS OF KETOACIDURIA AND KETOACIDEMIA IN DIABETICS: URINARY EXCRETION AND HEMATIC LEVELS OF ALPHA-KETOISOCAPROIC ACID, ALPHA-KETOISOVALERIC ACID, PYRUVIC ACID AND ALPHA-KETOGLUTARIC ACID].
Topics: Biomedical Research; Blood Chemical Analysis; Diabetes Mellitus; Hemiterpenes; Humans; Keto Acids; K | 1963 |
Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease.
Topics: Animals; Biological Transport; Brain; Carbon Dioxide; Disease Models, Animal; Energy Metabolism; Glu | 2003 |
Intrahippocampal administration of the alpha-keto acids accumulating in maple syrup urine disease provokes learning deficits in rats.
Topics: Animals; Avoidance Learning; Behavior, Animal; Hemiterpenes; Hippocampus; Injections, Intraventricul | 2004 |
Evidence that the branched-chain alpha-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex.
Topics: Actins; Animals; Animals, Newborn; Astrocytes; Brain; Cell Death; Cell Shape; Cells, Cultured; Cereb | 2004 |
alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats.
Topics: Animals; Antioxidants; Catalase; Cerebral Cortex; Disease Models, Animal; Free Radicals; Glutathione | 2005 |
Evidence that intracellular Ca2+ mediates the effect of alpha-ketoisocaproic acid on the phosphorylating system of cytoskeletal proteins from cerebral cortex of immature rats.
Topics: Animals; Autoradiography; Calcium; Calcium Channel Blockers; Calcium Channels; Calcium Radioisotopes | 2005 |
Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells.
Topics: Animals; Cell Line, Tumor; Cytoskeleton; Glial Fibrillary Acidic Protein; Glioma; Hemiterpenes; Keto | 2005 |
Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.
Topics: Animals; Antioxidants; Cell Line, Tumor; Cell Shape; Creatine; Creatine Kinase; Glioma; Hemiterpenes | 2006 |
Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.
Topics: Alanine; Animals; Cell Membrane; Cerebral Cortex; Enzyme Activation; Hemiterpenes; Keto Acids; Maple | 2007 |
Myelin proteins: degradation in rat brain initiated by metabolites causative of maple syrup urine disease.
Topics: Animals; Brain; Caproates; Electrophoresis, Polyacrylamide Gel; Humans; Keto Acids; Kinetics; Leucin | 1983 |
Intracellular levels and metabolism of leucine and alpha-ketoisocaproate in normal and maple syrup urine disease fibroblasts.
Topics: Amino Acids, Branched-Chain; Cells, Cultured; Decarboxylation; Fibroblasts; Humans; Keto Acids; Leuc | 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; Infan | 1984 |
Exchange transfusion in acute episodes of maple syrup urine disease. Studies on branched-chain amino and keto acids.
Topics: Amino Acids; Exchange Transfusion, Whole Blood; Female; Humans; Infant, Newborn; Infant, Newborn, Di | 1982 |
Blood and tissue branched-chain amino and alpha-keto acid concentrations: effect of diet, starvation, and disease.
Topics: Amino Acids, Branched-Chain; Animals; Diabetes Mellitus, Experimental; Diet; Hemiterpenes; Humans; I | 1981 |
Maple syrup urine disease: alpha-ketoisocaproate decarboxylation activity in different types of cultured amniotic fluid cells.
Topics: Amniotic Fluid; Cells, Cultured; Decarboxylation; Epithelium; Female; Fibroblasts; Humans; Keto Acid | 1981 |
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 Uri | 1995 |
Removal of branched-chain amino acids and alpha-ketoisocaproate by haemofiltration and haemodiafiltration.
Topics: Amino Acids, Branched-Chain; Animals; Cattle; Evaluation Studies as Topic; Hemodiafiltration; Hemofi | 1996 |
Effect of alpha-ketoisocaproate and leucine on the in vivo oxidation of glutamate and glutamine in the rat brain.
Topics: Animals; Brain; Caproates; Glutamic Acid; Glutamine; Keto Acids; Leucine; Male; Maple Syrup Urine Di | 1997 |
Assessment of whole body L-leucine oxidation by noninvasive L-[1-13C]leucine breath tests: a reappraisal in patients with maple syrup urine disease, obligate heterozygotes, and healthy subjects.
Topics: Adolescent; Adult; Breath Tests; Caproates; Carbon Dioxide; Carbon Isotopes; Child; Female; Heterozy | 1998 |
Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease.
Topics: Adolescent; Adult; Amino Acids, Branched-Chain; Child; Child, Preschool; Hemiterpenes; Humans; Isole | 1999 |
Branched chain amino acids induce apoptosis in neural cells without mitochondrial membrane depolarization or cytochrome c release: implications for neurological impairment associated with maple syrup urine disease.
Topics: Amino Acids, Branched-Chain; Animals; Apoptosis; Caspase 3; Caspases; Cell Respiration; Cells, Cultu | 2000 |
Chloride-dependent inhibition of vesicular glutamate uptake by alpha-keto acids accumulated in maple syrup urine disease.
Topics: Amino Acid Transport System X-AG; Animals; ATP-Binding Cassette Transporters; Biological Transport; | 2000 |
Inhibition of glutamate uptake into synaptic vesicles of rat brain by the metabolites accumulating in maple syrup urine disease.
Topics: Animals; Brain; Glutamic Acid; Hemiterpenes; Isoleucine; Keto Acids; Leucine; Male; Maple Syrup Urin | 2000 |
Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats.
Topics: Animals; Corpus Striatum; Excitatory Amino Acid Antagonists; GABA Agonists; Hemiterpenes; Keto Acids | 2001 |
Large neutral amino acids auto exchange when infused by microdialysis into the rat brain: implication for maple syrup urine disease and phenylketonuria.
Topics: Amino Acid Transport Systems, Neutral; Amino Acids, Cyclic; Amino Acids, Neutral; Animals; Binding, | 2002 |
Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex.
Topics: Animals; Animals, Newborn; Cerebral Cortex; Cytoskeleton; Dose-Response Relationship, Drug; Hemiterp | 2002 |
Prolongation of G1 and S phase in C-6 glioma cells treated with maple syrup urine disease metabolits. Morphologic and cell cycle studies.
Topics: Caproates; Cell Count; Cell Cycle; Cell Line; Humans; Interphase; Isoleucine; Keto Acids; Kinetics; | 1978 |
A convenient enzymatic method for the determination of 4-methyl-2-oxopentanoate in plasma: comparison with high performance liquid chromatographic analysis.
Topics: Alcohol Oxidoreductases; Chromatography, High Pressure Liquid; Humans; Keto Acids; Lacticaseibacillu | 1989 |
Analysis of maple syrup urine disease in cell culture: use of substrates.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Cells, Cultured; Fibroblasts; Hemiterpenes; Human | 1989 |
Simple method for rapid quantification of branched-chain 2-oxo acids in physiological fluids as quinoxalinol derivatives by high-performance liquid chromatography.
Topics: Amino Acids, Branched-Chain; Chromatography, High Pressure Liquid; Diet; Hemiterpenes; Humans; Keto | 1988 |