urea cycle

L-Ornithine hydrochloride ((S)-2,5-Diaminopentanoic acid) has an antifatigue effect by increasing the efficiency of energy consumption and promoting the excretion of ammonia. It is one of the key reactants in the urea cycle.

L-Ornithine ((S)-2, 5-diaminopentanoic acid) is a non-protein amino acid. L-Ornithine is mainly used in the urea cycle to remove excess nitrogen from the body. L-Ornithine is renoprotective. L-Ornithine has antifatigue effects.

2-Amino-5-ureidopentanoic acid (Citrulline) exists in the liver, is not a protein component, is an important intermediate in the human urea cycle, can be combined with ornithine, arginine to treat hyperammonemia.

4-Acetamidobutanoic acid (4-ACETAMIDOBUTYRIC ACID) can be found in blood, feces, and urine, as well as in human prostate tissue. 4-Acetamidobutanoic acid exists in all eukaryotes, ranging from yeast to humans. 4-Acetamidobutanoic acid is a GABA derivative, a product of the urea cycle and the metabolism of amino groups, and the product of NAD-linked aldehyde dehydrogenase (EC 1.2.1.3) (KEGG).

N-Acetyl-L-glutamic acid (Ac-L-Glu-OH) (NAcGlu) is an acetylated amino acid. NAcGlu is biosynthesized from glutamic acid and acetyl-CoA by the enzyme N-acetyl glutamate synthase (NAGS). NAcGlu activates carbamoyl phosphate synthetase in the urea cycle. A deficiency in N-acetyl glutamate synthase or a genetic mutation in the gene coding for the enzyme will lead to urea cycle failure in which ammonia is not converted to urea, but rather accumulated in the blood leading to the condition called Type I hyperammonemia.

4-Phenylbutyric acid (Benzenebutyric acid) is a histone deacetylase (HDAC) inhibitor and an endoplasmic reticulum stress (ERS) inhibitor. 4-Phenylbutyric acid can be used to treat urea cycle disorders.

TRPM7-IN-1 (compound SUD) is a benzamide-urea derivative and an effective inhibitor of TRPM7. This compound induces cell cycle arrest and apoptosis in MCF-7 and BGC-823 cells, reducing their migration capabilities. It decreases vimentin expression while increasing E-cadherin expression. TRPM7-IN-1 reduces TRPM7-like currents and inhibits TRPM7 expression by activating the PI3K/Akt signaling pathway. This compound shows potential as a therapeutic agent for reducing breast and gastric cancer metastasis by targeting TRPM7 expression and activity.

Argininosuccinic acid can be obtained during the urea cycle from citrulline, aspartate, and ATP by argininosuccinic acid synthetase.

L-Homocitrulline (Homocitrulline) is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders. The accumulation of carbamyl phosphate due to a depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of L-Homocitrulline and homoarginine in some cases.

Glutamate dehydrogenase NAD(P) (GLDH), a mitochondrial enzyme found in hepatocytes, renal tissue, brain, muscle, and intestinal cells, catalyzes the reversible oxidative deamination of glutamate to α-ketoglutarate (α-KG), playing a critical role in the urea cycle [1], and is frequently utilized in biochemical research.

Arginase (L-Arginine amidinase) is a key hydrolytic enzyme in the urea cycle, which hydrolyzes L-arginine into urea and L-ornithine. Arginase can be used for cancer research.

Glucagon significantly impacts protein and amino acid metabolism. It hinders the integration of amino acids into the proteins of the liver, muscle, and pancreas, augmenting nitrogen excretion and stimulating hepatic urea synthesis. Additionally, it elevates hepatic transaminase and urea cycle enzyme levels. Glucagon also enhances hepatic amino acid uptake, facilitating their conversion into liver glycogen, and reduces blood amino acid concentrations [1].

Carbamoyl phosphate dilithium salt acts as an anti-sickling agent and plays a significant role in the urea cycle. It modifies the terminal valine residues of hemoglobin S in red blood cells through formylation, thereby disrupting the sickling process. The lithium cations present in Carbamoyl phosphate dilithium salt contribute to its toxicity.

Orotic acid-13C,15N2 (monohydrate) is a marker used in the routine newborn screening for abnormalities in the urea cycle. Orotic acid can induce hepatic steatosis and liver enlargement in rats.

Oxaloacetic acid-13C4 is the 13C labeled form of oxaloacetic acid. Oxaloacetic acid (2-Oxosuccinic acid) serves as a metabolic intermediate involved in various pathways, including the citric acid cycle, gluconeogenesis, the urea cycle, the glyoxylate cycle, amino acid synthesis, and fatty acid synthesis.

L-Homocitrulline-d3 is the deuterated form of L-Homocitrulline. In the urea cycle pathway, L-Homocitrulline is metabolized into homoarginine by converting homoargininosuccinate. Metabolic irregularities involving L-Homocitrulline can result in lysinuric protein intolerance (LPI).

L-Citrulline (Standard) is available for quantitative analysis.) L-Citrulline is a non-essential amino acid involved in the nitric oxide cycle and the urea cycle, promotes vasodilatation, has antioxidant and anti-inflammatory properties, is orally available, and crosses the blood-brain barrier through LAT1. It is an agonist of eNOS (endothelial nitric oxide synthase), iNOS (inducible nitric oxide synthase), and mTORC1 (rapamycin targeting protein complex 1), and an inhibitor of SREBP-1 (sterol regulatory element binding protein 1).

D-Arginine (Standard) is a non-natural amino acid, used in biochemical research to study protein synthesis, signal transduction, nitrogen metabolism, and the urea cycle. D-Arginine (Standard) can be used for quantitative detection and research on metabolic pathways, neurological diseases, and drug development.

L-Ornithine hydrochloride (Standard) is a non-protein amino acid, which is mainly involved in the urea cycle, helps to remove excess ammoniacal nitrogen from the body, participates in a variety of metabolic processes and plays an important role in liver detoxification. As a standard, it can be used as a reference material for quantitative analysis in the study of amino acid metabolism and transport mechanism.

L-Homocitrulline (Standard) is a reference standard for research and analysis in studies involving L-Homocitrulline. L-Homocitrulline (Homocitrulline) is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders. The accumulation of carbamyl phosphate due to a depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of L-Homocitrulline and homoarginine in some cases.

2-Amino-5-ureidopentanoic acid (Standard) is a reference standard for research and analysis in studies involving 2-Amino-5-ureidopentanoic acid. 2-Amino-5-ureidopentanoic acid (Citrulline) exists in the liver, is not a protein component, is an important intermediate in the human urea cycle, can be combined with ornithine, arginine to treat hyperammonemia.