3-hydroxyisobutyric acid has been researched along with Amino Acid Metabolism Disorders, Inborn in 7 studies
3-hydroxyisobutyric acid: RN given refers to cpd without isomeric designation
3-hydroxyisobutyric acid : A 4-carbon, branched hydroxy fatty acid and intermediate in the metabolism of valine.
| Excerpt | Relevance | Reference |
|---|---|---|
| "3-Hydroxyisobutyric acid (3HiB) is an intermediate in the degradation of the branched-chain amino acid valine." | 1.62 | 3-Hydroxyisobutyrate dehydrogenase (HIBADH) deficiency-A novel disorder of valine metabolism. ( Biskup, S; Fobker, M; Hollenbeck, JC; Linden, M; Marquardt, T; Meyer, M; Och, U; Reunert, J; Rust, S; Sass, JO; Seelhöfer, A, 2021) |
| "3-hydroxyisobutyric aciduria is an organic aciduria with a poorly understood biochemical basis." | 1.38 | 3-Hydroxyisobutyrate aciduria and mutations in the ALDH6A1 gene coding for methylmalonate semialdehyde dehydrogenase. ( Atherton, AM; Garg, U; Sass, JO; Scott, D; Shield, JP; Smith, LD; Walter, M; Woods, CG, 2012) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 2 (28.57) | 18.7374 |
| 1990's | 2 (28.57) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 1 (14.29) | 24.3611 |
| 2020's | 2 (28.57) | 2.80 |
| Authors | Studies |
|---|---|
| Sasarman, F | 1 |
| Ferdinandusse, S | 1 |
| Sinasac, DS | 1 |
| Fung, E | 1 |
| Sparkes, R | 1 |
| Reeves, M | 1 |
| Rombough, C | 1 |
| Sass, JO | 3 |
| Voit, R | 1 |
| Ruiter, JPN | 1 |
| Koster, J | 1 |
| Waterham, HR | 1 |
| Pasquini, E | 1 |
| Donati, MA | 1 |
| Marquardt, T | 2 |
| Wanders, RJA | 1 |
| Al-Hertani, W | 1 |
| Meyer, M | 1 |
| Hollenbeck, JC | 1 |
| Reunert, J | 1 |
| Seelhöfer, A | 1 |
| Rust, S | 1 |
| Fobker, M | 1 |
| Biskup, S | 1 |
| Och, U | 1 |
| Linden, M | 1 |
| Walter, M | 1 |
| Shield, JP | 1 |
| Atherton, AM | 1 |
| Garg, U | 1 |
| Scott, D | 1 |
| Woods, CG | 1 |
| Smith, LD | 1 |
| Congdon, PJ | 1 |
| Haigh, D | 1 |
| Smith, R | 2 |
| Green, A | 2 |
| Pollitt, RJ | 2 |
| Yoshida, I | 1 |
| Ko, FJ | 1 |
| Nyhan, WL | 1 |
| Wolff, J | 1 |
| Barshop, B | 1 |
| Sweetman, L | 1 |
1 review available for 3-hydroxyisobutyric acid and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
[3-Hydroxyisobutyric aciduria (3-hydroxyisobutyric acid dehydrogenase deficiency)].
Topics: Amino Acid Metabolism, Inborn Errors; Diagnosis, Differential; Humans; Hydroxybutyrates; Prognosis; | 1998 |
6 other studies available for 3-hydroxyisobutyric acid and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
3-Hydroxyisobutyric acid dehydrogenase deficiency: Expanding the clinical spectrum and quantitation of D- and L-3-Hydroxyisobutyric acid by an LC-MS/MS method.
Topics: Amino Acid Metabolism, Inborn Errors; Chromatography, Liquid; Humans; Hydroxybutyrates; Oxidoreducta | 2022 |
3-Hydroxyisobutyrate dehydrogenase (HIBADH) deficiency-A novel disorder of valine metabolism.
Topics: Alcohol Oxidoreductases; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Diagnosis, Differen | 2021 |
3-Hydroxyisobutyrate aciduria and mutations in the ALDH6A1 gene coding for methylmalonate semialdehyde dehydrogenase.
Topics: Amino Acid Metabolism, Inborn Errors; Consanguinity; DNA Mutational Analysis; Female; Fibroblasts; H | 2012 |
Hypermethioninaemia and 3-hydroxyisobutyric aciduria in an apparently healthy baby.
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Aminoisobutyric Acids; Humans; Hydroxybutyrates; Infa | 1981 |
3-Hydroxyisobutyric aciduria: an inborn error of valine metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Child; Humans; Hydroxybutyrates; Male; Valine | 1991 |
Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes.
Topics: Alanine; Aldehyde Oxidoreductases; Amino Acid Metabolism, Inborn Errors; Aminoisobutyric Acids; beta | 1985 |