Page last updated: 2024-10-06

Argininosuccinic Aciduria

Proteins (13)

ProteinSynonymsTaxonomy
Aspartate aminotransferase, mitochondrialmAspAT; EC 2.6.1.1; EC 2.6.1.7; Fatty acid-binding protein; FABP-1; Glutamate oxaloacetate transaminase 2; Kynurenine aminotransferase 4; Kynurenine aminotransferase IV; Kynurenine--oxoglutarate transaminase 4; Kynurenine--oxoglutarate transaminase IV; PlHomo sapiens (human)
Neutral amino acid transporter B(0)ATB(0); Baboon M7 virus receptor; RD114/simian type D retrovirus receptor; Sodium-dependent neutral amino acid transporter type 2; Solute carrier family 1 member 5Homo sapiens (human)
Glutaminase liver isoform, mitochondrialGLS; EC 3.5.1.2; L-glutaminase; L-glutamine amidohydrolaseHomo 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)
Neutral amino acid transporter AAlanine/serine/cysteine/threonine transporter 1; ASCT-1; SATT; Solute carrier family 1 member 4Homo sapiens (human)
Carbamoyl-phosphate synthase [ammonia], mitochondrialEC 6.3.4.16; Carbamoyl-phosphate synthetase I; CPSase IHomo sapiens (human)
Arginase-1EC 3.5.3.1; Liver-type arginase; Type I arginaseHomo sapiens (human)
Ornithine transcarbamylase, mitochondrialOTCase; EC 2.1.3.3; Ornithine carbamoyltransferase, mitochondrialHomo sapiens (human)
Argininosuccinate synthaseEC 6.3.4.5; Citrulline--aspartate ligaseHomo sapiens (human)
Glutamate dehydrogenase 1, mitochondrialGDH 1; EC 1.4.1.3Homo sapiens (human)
Calcium-binding mitochondrial carrier protein Aralar1Mitochondrial aspartate glutamate carrier 1; Solute carrier family 25 member 12Homo sapiens (human)
Argininosuccinate lyaseASAL; EC 4.3.2.1; ArginosuccinaseHomo sapiens (human)
Mitochondrial ornithine transporter 1Solute carrier family 25 member 15Homo sapiens (human)

Compounds (21)

CompoundDescription
phosphoric acidconcise etchant is 37% H3PO4
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
ureaA compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids.
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
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.
NADH
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.
glutamineA non-essential amino acid present abundantly throughout the body and is involved in many metabolic processes. It is synthesized from GLUTAMIC ACID and AMMONIA. It is the principal carrier of NITROGEN in the body and is an important energy source for many cells.
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.
ornithineAn amino acid produced in the urea cycle by the splitting off of urea from arginine.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
Argininosuccinic AcidThis amino acid is formed during the urea cycle from citrulline, aspartate and ATP. This reaction is catalyzed by argininosuccinic acid synthetase.
carbamyl phosphateThe monoanhydride of carbamic acid with PHOSPHORIC ACID. It is an important intermediate metabolite and is synthesized enzymatically by CARBAMYL-PHOSPHATE SYNTHASE (AMMONIA) and CARBAMOYL-PHOSPHATE SYNTHASE (GLUTAMINE-HYDROLYZING).
alpha-ketoglutaric acid
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
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.
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