Pathways > Pyruvate Carboxylase Deficiency
Page last updated: 2024-10-06
Pyruvate Carboxylase Deficiency
Proteins (5)
| Protein | Synonyms | Taxonomy |
|---|
| Serine--pyruvate aminotransferase | SPT; EC 2.6.1.51; Alanine--glyoxylate aminotransferase; AGT; 2.6.1.44 | Homo sapiens (human) |
| Alanine aminotransferase 1 | ALT1; EC 2.6.1.2; Glutamate pyruvate transaminase 1; GPT 1; Glutamic--alanine transaminase 1; Glutamic--pyruvic transaminase 1 | Homo sapiens (human) |
| Alanine--tRNA ligase, mitochondrial | EC 6.1.1.7; Alanyl-tRNA synthetase; AlaRS | Homo sapiens (human) |
| Pyruvate carboxylase, mitochondrial | EC 6.4.1.1; Pyruvic carboxylase; PCB | Homo sapiens (human) |
| Mitochondrial pyruvate carrier 1 | Brain protein 44-like protein | Homo sapiens (human) |
Compounds (12)
| Compound | Description |
|---|
| 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) |
| Orthophosphate | |
| glutamic acid | A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM. |
| Adenosine Triphosphate | An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter. |
| adenosine diphosphate | Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position. |
| adenosine monophosphate | Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position. |
| hydrogen carbonate | |
| alpha-ketoglutaric acid | |
| alanine | A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM. |
| glyoxylic acid | RN given refers to parent cpd |
| 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. |
| pyrophosphate | |