alpha-ketoisovalerate has been researched along with BCKD Deficiency in 21 studies
alpha-ketoisovalerate: RN given refers to parent cpd
3-methyl-2-oxobutanoate : A 2-oxo monocarboxylic acid anion that is the conjugate base of 3-methyl-2-oxobutanoic acid, arising from deprotonation of the carboxy group.
3-methyl-2-oxobutanoic acid : A 2-oxo monocarboxylic acid that is the 2-oxo derivative of isovaleric acid.
| 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) |
| " 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) |
| "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) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 7 (33.33) | 18.7374 |
| 1990's | 3 (14.29) | 18.2507 |
| 2000's | 11 (52.38) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| PICCARDO, MG | 1 |
| LUZIETTI, L | 1 |
| CAPURSO, L | 1 |
| Sgaravatti, AM | 1 |
| Rosa, RB | 1 |
| Schuck, PF | 2 |
| Ribeiro, CA | 1 |
| Wannmacher, CM | 4 |
| Wyse, AT | 1 |
| Dutra-Filho, CS | 4 |
| Wajner, M | 11 |
| de Castro Vasques, V | 1 |
| de Boer, MA | 1 |
| Diligenti, F | 1 |
| Brinco, F | 1 |
| Mallmann, F | 1 |
| Mello, CF | 1 |
| Funchal, C | 4 |
| Gottfried, C | 3 |
| De Almeida, LM | 1 |
| Pessoa-Pureur, R | 4 |
| Bridi, R | 1 |
| Braun, CA | 1 |
| Zorzi, GK | 1 |
| Lissi, EG | 1 |
| Dos Santos, AQ | 1 |
| Jacques-Silva, MC | 2 |
| Zamoner, A | 1 |
| Santos, AQ | 1 |
| Wajner, A | 1 |
| Bürger, C | 1 |
| de Souza Wyse, AT | 1 |
| Snyderman, SE | 1 |
| Goldstein, F | 1 |
| Sansaricq, C | 1 |
| Norton, PM | 1 |
| Chuang, DT | 3 |
| Ku, LS | 2 |
| Cox, RP | 3 |
| Hutson, SM | 1 |
| Harper, AE | 1 |
| Schlottfeldt, JL | 1 |
| Ckless, K | 1 |
| Chuang, JL | 1 |
| Davie, JR | 1 |
| Chinsky, JM | 1 |
| Wynn, RM | 1 |
| Schadewaldt, P | 2 |
| Hammen, HW | 1 |
| Ott, AC | 1 |
| Wendel, U | 2 |
| 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 |
| de Lima Pelaez, P | 1 |
| Vivian, L | 1 |
| Oliveira Loureiro, S | 1 |
| de Freitas Miranda, R | 1 |
| Beck, K | 2 |
| Radeck, W | 1 |
| Staib, W | 1 |
21 other studies available for alpha-ketoisovalerate and BCKD Deficiency
| Article | Year |
|---|---|
[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 |
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 |
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 |
Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Cells, Cultured; Hemiterpenes; Humans; Keto Acids | 1982 |
Biochemical basis of thiamin-responsive maple syrup urine disease.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Cells, Cultured; Decarboxylation; Female; Fibrobl | 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 |
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 |
Molecular and biochemical basis of intermediate maple syrup urine disease. Occurrence of homozygous G245R and F364C mutations at the E1 alpha locus of Hispanic-Mexican patients.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Base Sequence; Blotting, Northern; Blotting, West | 1995 |
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 |
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 |
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 |
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 |