alpha-ketoisovalerate has been researched along with Maple Syrup Urine Disease 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.
Maple Syrup Urine Disease: An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a maple syrup odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)
| 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 Maple Syrup Urine Disease
| 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 |