excretion

3-Hydroxyisovaleric acid (Beta-Hydroxyisovaleric acid) is an endogenous metabolite excreted in the urine and can serve as an early and sensitive indicator of biotin deficiency.

Penicillamine (Artamine), possessing antineoplastic properties, induces apoptosis by a p53-mediated mechanism and inhibits angiogenesis by chelating with copper, a cofactor for angiogenesis. Artamine is a beta dimethyl analog of the amino acid cysteine. As a degradation product of penicillin antibiotics, Artamine chelates with heavy metals and increases their urinary excretion.

Hydrocortisone (Cortisol) is a glucocorticoid hormone secreted by the adrenocortex. Hydrocortisone agonizes the glucocorticoid receptor, which promotes proteolytic metabolism, gluconeogenesis, capillary wall stabilization, and renal calcium excretion, as well as suppressing immune and inflammatory responses.

Cholic Acid (Cholate) is a major primary bile acid produced in the liver and usually conjugated with glycine or taurine. Cholic acid facilitates fat absorption and cholesterol excretion.

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.

Cholic acid sodium (SodiumCholate) exhibits a strong spermicidal and antiviral [anti-human immunodeficiency virus (HIV)-1] activity.

Pyrrole-2-carboxylic acid (Minaline) was first identified as a degradation product of sialic acids, then as a derivative of the oxidation of the D-hydroxyproline isomers by mammalian D-amino acid oxidase. The latter relationship results from the lability of the direct oxidation product, A'-pyrroline-4-hydroxy-2-carboxylic acid, which loses water spontaneously to form the pyrrole. A similar reaction is catalyzed by the more specific allohydroxy-D-proline oxidase of Pseudomonas. In whole animal observations, pyrrole-2-carboxylate (PCA) ' was identified in rat or human urine after administration of the D-isomers of hydroxyproline, a finding ascribable to the action of D-amino acid oxidase. Urinary excretion of N-(pyrrole-2-carboxyl) glycine has been reported in a 5-year-old affected with type II hyperprolinemia; The child has mild developmental delay, recurrent seizures of the grand mal type and EEG alterations.

NSC-16590 (2-Aminoisobutyric acid) is a nonprotein amino acid (is an end product of pyrimidine Metabolism) excreted in the urine of about 5% of healthy individuals, and high excretion is an autosomal recessive phenotype.

Trans-2-butene-1,4-dicarboxylic acid (3-Hexenedioic Acid) is a normal human unsaturated dicarboxylic acid metabolite with increased excretion in patients with Dicarboxylic aciduria caused by fatty acid metabolism disorders The urinary excretion of Trans-2-butene-1,4-dicarboxylic acid is increased in conditions of augmented mobilization of fatty acids or inhibited fatty acid oxidation.

Cholic acid sodium hydrate is a natural and orally active bile acid that is synthesized primarily by the liver and secreted into the bile, where it plays an important role in the digestive process, aiding in the absorption of fats by emulsifying them, as well as participating in the excretion of cholesterol and regulating the metabolism of bile acids.

N-Isovaleroylglycine (Isovaleroylglycine) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases, the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine. N-Isovaleroylglycine is a byproduct of the catabolism of the amino acid leucine. Accumulation of N-Isovaleroylglycine occurs in Isovaleric Acidemia (IVA).

L-Ornithine ((S)-2,5-Diaminopentanoic acid) exhibits an anti-fatigue function by promoting ammonia excretion and enhancing energy consumption efficiency.

Tetrahydro-11-deoxy cortisol (THS) is the primary urinary metabolite of 11-deoxycortisol. Urinary excretion of THS is elevated in patients with 11β-hydroxylase deficiency, a condition resulting from mutations in the cytochrome P450 (CYP) isoform CYP11B1. Urinary levels of THS are also elevated in patients with adrenocortical carcinoma (ACC) and adrenocortical adenoma (ACA) but are higher in patients with ACC compared to ACA.

L-Glyceric acid sodium, a urinary metabolite, is predominantly found in individuals with the rare inherited metabolic disorder L-glyceric aciduria. This compound is a diagnostic tool for identifying primary hyperoxaluria type 2 (PH2) and distinguishing it from primary hyperoxaluria type 1 (PH1) based on its excretion pattern.

4-Hydroxyphenylpyruvic acid is an enzyme inhibitor.4-Hydroxyphenylpyruvic acid (4-HPPA) is a keto acid that is involved in the tyrosine catabolism pathway. It is a product of the enzyme (R)-4-hydroxyphenyllactate dehydrogenase (EC 1.1.1.222) and is formed during tyrosine metabolism. The conversion from tyrosine to 4-HPPA is catalyzed by tyrosine aminotransferase. Additionally, 4-HPPA can be converted to homogentisic acid which is one of the precursors to ochronotic pigment. The enzyme 4-hydroxyphenylpyruvic acid dioxygenase (HPD) catalyzes the reaction that converts 4-hydroxyphenylpyruvic acid to homogentisic acid. A deficiency in the catalytic activity of HPD is known to lead to tyrosinemia type III, an autosomal recessive disorder characterized by elevated levels of blood tyrosine and massive excretion of tyrosine derivatives into urine. It has been shown that hawkinsinuria, an autosomal dominant disorder characterized by the excretion of 'hawkinsin,' may also be a result of HPD deficiency . Moreover, 4-hydroxyphenylpyruvic acid is also found to be associated in phenylketonuria, which is also an inborn error of metabolism. There are two isomers of HPPA, specifically 4HPPA and 3HPPA, of which 4HPPA is the most common.

N-(3-Phenylpropionyl)glycine (Phenylpropionylglycine) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. Acyl glycines are produced through the action of glycine N-acyltransferase which is an enzyme that catalyzes the chemical reaction:acyl-CoA + glycine < - > CoA + N-acylglycineThe detection of phenylpropionylglycine in urine after an oral load of phenylpropionic acid can be used to diagnose deficiency of medium-chain acyl-CoA dehydrogenase, a frequent and treatable metabolic defect.

1. Coptisine (Coptisin) treatment increases cell viability based on its reversal effect on the enhanced activity of Indoleamine 2, 3-dioxygenase . 2. Coptisine treats myocardial I R likely through suppressing myocardial apoptosis and inflammation by inhibiting the Rho ROCK pathway. 3. Coptisine is a potential anti-osteosarcoma drug candidate, via exerting a strong anti-osteosarcoma effect with very low toxicity . 4. Coptisine with a high dosage could inhibit cholesterol synthesis via suppressing the HMGCR expression and promoting the use and excretion of cholesterol via up-regulating LDLR and CYP7A1 expression. 5. Coptisine suppresses adhesion, migration and invasion of MDA-MB-231 breast cancer cells in vitro, the down-regulation of MMP-9 in combination with the increase of TIMP-1 possibly contributing to the anti-metastatic function for breast cancer.