Page last updated: 2024-10-06

Pyruvate Carboxylase Deficiency

Proteins (5)

ProteinSynonymsTaxonomy
Serine--pyruvate aminotransferaseSPT; EC 2.6.1.51; Alanine--glyoxylate aminotransferase; AGT; 2.6.1.44Homo sapiens (human)
Alanine aminotransferase 1ALT1; EC 2.6.1.2; Glutamate pyruvate transaminase 1; GPT 1; Glutamic--alanine transaminase 1; Glutamic--pyruvic transaminase 1Homo sapiens (human)
Alanine--tRNA ligase, mitochondrialEC 6.1.1.7; Alanyl-tRNA synthetase; AlaRSHomo sapiens (human)
Pyruvate carboxylase, mitochondrialEC 6.4.1.1; Pyruvic carboxylase; PCBHomo sapiens (human)
Mitochondrial pyruvate carrier 1Brain protein 44-like proteinHomo sapiens (human)

Compounds (12)

CompoundDescription
pyruvic acidAn 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 acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
Adenosine TriphosphateAn 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 diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
hydrogen carbonate
alpha-ketoglutaric acid
alanineA 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 acidRN given refers to parent cpd
oxaloacetic acidA 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