| Thiamine Triphosphate | 3-((4-Amino-2-methyl-5-pyrimidinyl)methyl)-4-methyl-5-(4,6,8,8-tetrahydroxy-3,5,7-trioxa-4,6,8-triphosphaoct-1-yl)thiazolium hydroxide, inner salt, P,P',P''-trioxide. The triphosphate ester of thiamine. In Leigh's disease, this compound is present in decreased amounts in the brain due to a metabolic block in its formation. |
| Succinate | |
| alpha-glycerophosphoric acid | |
| thiamine | 3-((4-Amino-2-methyl-5-pyrimidinyl)methyl)-5-(2- hydroxyethyl)-4-methylthiazolium chloride. |
| Pyruvic Acid | An intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed) |
| alpha-Ketoglutarate | |
| glyceraldehyde 3-phosphate | An aldotriose which is an important intermediate in glycolysis and in tryptophan biosynthesis. |
| isocitrate | |
| Glucose-6-Phosphate | An ester of glucose with phosphoric acid, made in the course of glucose metabolism by mammalian and other cells. It is a normal constituent of resting muscle and probably is in constant equilibrium with fructose-6-phosphate. (Stedman, 26th ed) |
| Serine | A non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from GLYCINE or THREONINE. It is involved in the biosynthesis of PURINES; PYRIMIDINES; and other amino acids. |
| dihydroxyacetone phosphate | An important intermediate in lipid biosynthesis and in glycolysis. |
| oxaloacetic acid | A dicarboxylic acid ketone that is an important metabolic intermediate of the CITRIC ACID CYCLE. It can be converted to ASPARTIC ACID by ASPARTATE TRANSAMINASE. |
| acetyl coenzyme a | Acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent. |
| glucose-1-phosphate | RN given refers to (alpha-D-Glc)-isomer |