thiamine pyrophosphate has been researched along with BCKD Deficiency in 6 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 5 (83.33) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 1 (16.67) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Awad, N; Chuang, DT; Chuang, JL; Li, J; Mandel, H; Moss, CC; Song, JL; Wynn, RM | 1 |
| Chuang, DT; Cox, RP; Ku, LS | 1 |
| Danner, DJ; Elsas, LJ | 1 |
| Goedde, HW; Singh, S; Willers, I | 1 |
| Danner, DJ; Elsas, LJ; Lemmon, SK; Wheeler, FB | 1 |
| Elsas, LJ; Pask, BA; Perl, DP; Truster, S; Wheeler, FB | 1 |
1 review(s) available for thiamine pyrophosphate and BCKD Deficiency
| Article | Year |
|---|---|
The role of thiamin in maple syrup urine disease.
Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Amino Acids, Branched-Chain; Cells, Cultured; Child; Child, Preschool; Dietary Proteins; Drug Stability; Female; Fibroblasts; Hot Temperature; Humans; Infant; Keto Acids; Ketone Oxidoreductases; Kinetics; Lymphocytes; Male; Maple Syrup Urine Disease; Mitochondria, Liver; Multienzyme Complexes; Protein Conformation; Thiamine; Thiamine Pyrophosphate | 1982 |
5 other study(ies) available for thiamine pyrophosphate and BCKD Deficiency
| 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 Relationship, Drug; Homozygote; Humans; Israel; Kinetics; Maple Syrup Urine Disease; Methylamines; Models, Chemical; Models, Molecular; Mutation; Phenotype; Protein Binding; Protein Folding; Protein Structure, Tertiary; Recombinant Proteins; Thermodynamics; Thiamine; Thiamine Pyrophosphate | 2004 |
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; Ketone Oxidoreductases; Kinetics; Maple Syrup Urine Disease; Multienzyme Complexes; Pyruvate Dehydrogenase Complex; Substrate Specificity; Thiamine; Thiamine Pyrophosphate | 1982 |
Heterogeneity in maple syrup urine disease: aspects of cofactor requirement and complementation in cultured fibroblasts.
Topics: Caproates; Carboxy-Lyases; Cells, Cultured; Coenzyme A; Decarboxylation; Fibroblasts; Humans; Hybrid Cells; Keto Acids; Maple Syrup Urine Disease; NAD; Thiamine Pyrophosphate; Valerates | 1977 |
In vivo and in vitro response of human branched chain alpha-ketoacid dehydrogenase to thiamine and thiamine pyrophosphate.
Topics: Fibroblasts; Hot Temperature; Humans; In Vitro Techniques; Keto Acids; Ketone Oxidoreductases; Leukocytes; Male; Maple Syrup Urine Disease; Mitochondria; Thiamine; Thiamine Pyrophosphate; Valerates | 1978 |
Classical maple syrup urine disease: cofactor resistance.
Topics: Amino Acids; Carbon Isotopes; Cells, Cultured; Coenzyme A; Coenzymes; Female; Fibroblasts; Flavin-Adenine Dinucleotide; Genetics, Medical; Humans; Infant, Newborn; Isoleucine; Keto Acids; Leucine; Male; Maple Syrup Urine Disease; NAD; Pyridoxal Phosphate; Pyruvates; Skin; Thiamine Pyrophosphate; Thioctic Acid; Valine | 1972 |