metabolic disorder

Entacapone is a potent and specific peripheral catechol-O-methyltransferase (COMT) inhibitor with an IC50 of 151 nM. Entacapone is also a potential obesity-related gene (FTO) inhibitor that can inhibit FTO demethylation activity and be used in the study of metabolic diseases.

Trimyristin (Tritetradecanoin) is primarily located in the membrane (predicted from logP) and adiposome. glyceryl trimyristate exists in all eukaryotes, ranging from yeast to humans. In humans, glyceryl trimyristate is involved in the metabolic disorder called de novo triacylglycerol biosynthesis glyceryl trimyristate pathway.

N-Acetyl-L-arginine (Ac-Arg-OH), an N-acyl-L-alpha-amino acid with a strong basic nature (based on its pKa), has elevated serum levels in hyperargininemic patients and is associated with several diseases, including uremia and colorectal cancer. In untreated hyperargininemic patients, it reaches its highest levels in cerebrospinal fluid. It has also been detected in apples and loquats, suggesting it could be a potential biomarker for consumption of these foods. Additionally, a low-arginine diet combined with sodium benzoate therapy leads to a marked decrease in plasma N-acetyl-L-arginine concentrations.

Nequinate is an anticoccidial agent, preventing coccidiosis .

Ethyl cinnamate has antifungal, and vasorelaxant effects. Ethyl cinnamate can lead to the damage of cell membrane system and metabolic disorder through inducing lipid peroxidation via initiating ROS overproduction. Ethyl cinnamate can inhibit the tonic contractions induced by high K+ and phenylephrine (PE) in a concentration-dependent manner, with respective IC50 values of 0.30 mM and 0.38 mM.

TXNIP-IN-1 is an inhibitor of the TXNIP-TRX complex and used in the research of TXNIP-TRX complex associated metabolic disorder (diabetes), cardiovascular disease, or inflammatory disease.

Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid. They are metabolic precursors to sulfatides , found primarily in nerve tissues, and are the main glycosphingolipids in the central nervous system. Galactosylcerebrosides are involved in a multitude of cellular processes including cell agglutination, cellular signaling in glycosynapses, cellular development, and activation of T cells. They accumulate in globoid cells in the brain of patients with Krabbe disease, a disorder characterized by a deficiency in galactosylcerebrosidase activity. This product is a mixture of isolated bovine hydroxy galactosylcerebrosides.

Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid. They are metabolic precursors to sulfatides , found primarily in nerve tissues, and are the main glycosphingolipids in the central nervous system. Galactosylcerebrosides are involved in a multitude of cellular processes including cell agglutination, cellular signaling in glycosynapses, cellular development, and activation of T cells. They accumulate in globoid cells in the brain of patients with Krabbe disease, a disorder characterized by a deficiency in galactosylcerebrosidase activity. This product is a mixture of isolated bovine non-hydroxy galactosylcerebrosides.

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.

Tiludronate (Tiludronic Acid) disodium hemihydrate is an orally active bisphosphonate compound used primarily in metabolic bone disorder research. It functions as an osteoregulator and acts as a potent inhibitor of the osteoclast vacuolar H+ -ATPase, possessing antiresorptive and anti-inflammatory properties [4].

2-Methylsuccinic acid (Pyrotartaric Acid) is a normal metabolite found in human fluids. Increased urinary levels of 2-Methylsuccinic acid (Pyrotartaric Acid) (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy, a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay, hyperlactic acidemia, recurrent petechiae, orthostatic acrocyanosis, and chronic diarrhea.

JBSNF-000028 TFA, a nicotinamide N-methyltransferase (NNMT) inhibitor, exhibits oral activity with inhibitory concentration 50 (IC50) values of 0.033 μM, 0.19 μM, and 0.21 μM for human NNMT (hNNMT), monkey NNMT (mkNNMT), and mouse NNMT (mNNMT) respectively. It is applicable in metabolic disorder research [1].

PI5P4Kα-IN-1 (Compound 13) is a phosphatidylinositol 5-phosphate 4-kinase (PI5P4K) inhibitor with IC50 values of 2 μM for PI5P4Kα and 9.4 μM for PI5P4Kβ, potentially applicable in cancer, metabolic, and immunological disorder studies [1].

3-Methylhistidine belongs to the class of organic compounds known as histidine and derivatives. Histidine and derivatives are compounds containing cysteine or a derivative thereof resulting from reaction of cysteine at the amino group or the carboxy group

Propionicacid-d5 is a deuterated compound of Propionicacid. Propionicacid has a CAS number of 79-09-4. Propionic acid (PA) is widely used as an antifungal agent in food. It is present naturally at low levels in dairy products and occurs ubiquitously, together with other short-chain fatty acids (SCFA), in the gastro-intestinal tract of humans and other mammals as an end-product of the microbial digestion of carbohydrates. It has significant physiological activity in animals. PA is irritant but produces no acute systemic effects and has no demonstrable genotoxic potential.Propionic aciduria is one of the most frequent organic acidurias, a disease that comprise many various disorders. The outcome of patients born with Propionic aciduria (genetic disorder) is poor intellectual development patterns, with 60% having an IQ less than 75 and requiring special education. Successful liver and or renal transplantations, in a few patients, have resulted in better quality of life but have not necessarily prevented neurological and various visceral complications. These results emphasize the need for permanent metabolic follow-up whatever the therapeutic strategy. Decreased early mortality, less severe symptoms at diagnosis, and more favorable short-term neurodevelopmental outcome were recorded in patients identified through expanded newborn screening.

Propionic-2,2-d2 acid is a deuterated compound of Propionic acid. Propionic acid has a CAS number of 79-09-4. Propionic acid (PA) is widely used as an antifungal agent in food. It is present naturally at low levels in dairy products and occurs ubiquitously, together with other short-chain fatty acids (SCFA), in the gastro-intestinal tract of humans and other mammals as an end-product of the microbial digestion of carbohydrates. It has significant physiological activity in animals. PA is irritant but produces no acute systemic effects and has no demonstrable genotoxic potential.Propionic aciduria is one of the most frequent organic acidurias, a disease that comprise many various disorders. The outcome of patients born with Propionic aciduria (genetic disorder) is poor intellectual development patterns, with 60% having an IQ less than 75 and requiring special education. Successful liver and or renal transplantations, in a few patients, have resulted in better quality of life but have not necessarily prevented neurological and various visceral complications. These results emphasize the need for permanent metabolic follow-up whatever the therapeutic strategy. Decreased early mortality, less severe symptoms at diagnosis, and more favorable short-term neurodevelopmental outcome were recorded in patients identified through expanded newborn screening.

21-Deoxy Cortisone (21-Desoxycortisone; NSC 38722) is a corticosteroid metabolite of 11-ketoprogesterone. It is generated from 11-ketoprogesterone by the cytochrome P450 (CYP) isoform CYP17A1, and can also be produced by oxidation via 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2) from 21-deoxycorticosterone. In patients with congenital adrenal hyperplasia, a metabolic disorder characterized by a deficiency of 21-hydroxylase, levels of 21-Deoxy Cortisone are elevated.

(R)-3-Hydroxyisobutyric acid sodium is a biochemical marker of the metabolic disorder, 3-hydroxyisobutyric aciduria, characterized by increased concentrations in urine.