trimethyloxamine has been researched along with Maple Syrup Urine Disease in 2 studies
trimethyloxamine: used in manufacture of quaternary ammonium cpds; insect attractant; warming agent for gas; oxidant; structure
trimethylamine N-oxide : A tertiary amine oxide resulting from the oxidation of the amino group of trimethylamine.
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 |
|---|---|---|
| "Maple syrup urine disease is caused by deficiency in the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex." | 1.31 | Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease. ( Chuang, DT; Song, JL, 2001) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 2 (100.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Chuang, JL | 1 |
| Wynn, RM | 1 |
| Moss, CC | 1 |
| Song, JL | 2 |
| Li, J | 1 |
| Awad, N | 1 |
| Mandel, H | 1 |
| Chuang, DT | 2 |
2 other studies available for trimethyloxamine and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Structural and biochemical basis for novel mutations in homozygous Israeli maple syrup urine disease patients: a proposed mechanism for the thiamin-responsive phenotype.
Topics: Alleles; Blotting, Western; Centrifugation, Density Gradient; DNA, Complementary; Dose-Response Rela | 2004 |
Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Dimerization; Enzyme Stability; Humans; Ketone Ox | 2001 |