Primary Hyperoxaluria Type I

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)

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