alpha-ketoadipic acid has been researched along with Maple Syrup Urine Disease in 1 studies
2-oxoadipic acid : An oxo dicarboxylic acid that is adipic acid substituted by an oxo group at position 2.
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 |
|---|---|---|
| "There are several organic acid disorders that require information on alpha-ketoacids, such as maple syrup urine disease or alpha-ketoadipic acidemia." | 3.71 | Gas chromatographic-mass spectrometric screening for organic acidemias using dried urine filter paper: determination of alpha-ketoacids. ( Fu, X; Iga, M; Kimura, M; Yamaguchi, S, 2001) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (100.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Fu, X | 1 |
| Kimura, M | 1 |
| Iga, M | 1 |
| Yamaguchi, S | 1 |
1 other study available for alpha-ketoadipic acid and Maple Syrup Urine Disease
| Article | Year |
|---|---|
Gas chromatographic-mass spectrometric screening for organic acidemias using dried urine filter paper: determination of alpha-ketoacids.
Topics: Acids; Adipates; Gas Chromatography-Mass Spectrometry; Humans; Maple Syrup Urine Disease; Paper; Ref | 2001 |