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Showing 261 - 280 of 55735 compounds

Compound ID

Compound Description

Pathway Class

Pathways

PW_C000605

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Glycogen

Glycogen is a highly-branched polymer of about 30,000 glucose residues and has a molecular weight between 106 and 107 daltons (4.8 million approx.). Most of Glc units are linked by alpha-1,4 glycosidic bonds, approximately 1 in 12 Glc residues also makes -1,6 glycosidic bond with a second Glc which results in the creation of a branch. Glycogen only has one reducing end and a large number of non-reducing ends with a free hydroxyl group at carbon 4. The glycogen granules contain both glycogen and the enzymes of glycogen synthesis (glycogenesis) and degradation (glycogenolysis). The enzymes are nested between the outer branches of the glycogen molecules and act on the non-reducing ends. Therefore, the many non-reducing end-branches of glycogen facilitate its rapid synthesis and breakdown. In hypoglycemia caused by excessive insulin, liver glycogen levels are high, but the high insulin level prevents the glycogenolysis necessary to maintain normal blood sugar levels. Glucagon is a common treatment for this type of hypoglycemia. Glycogen is a polysaccharide that is the principal storage form of glucose (Glc) in animal and human cells. Glycogen is found in the form of granules in the cytosol in many cell types. Hepatocytes (liver cells) have the highest concentration of it - up to 8% of the fresh weight in well fed state, or 100 to 120 g in an adult - giving liver a distinctive, 'starchy taste'. In the muscles, glycogen is found in a much lower concentration (1% of the muscle mass), but the total amount exceeds that in liver. Small amounts of glycogen are found in the kidneys, and even smaller amounts in certain glial cells in the brain and white blood cells.
Drug Metabolism
Drug Action

PW_C000608

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Lithocholic acid

Lithocholic acid, also known as 3α-hydroxy-5β-cholan-24-oic acid or LCA, is a secondary bile acid. It is formed from chenodeoxycholate by bacterial action, and is usually conjugated with glycine or taurine. It acts as a detergent to solubilize fats for absorption and is itself absorbed. It is used as cholagogue and choleretic. Bile acids are steroid acids found predominantly in the bile of mammals. The distinction between different bile acids is minute, and depends only on the presence or absence of hydroxyl groups on positions 3, 7, and 12. Bile acids are physiological detergents that facilitate excretion, absorption, and transport of fats and sterols in the intestine and liver. Bile acids are also steroidal amphipathic molecules derived from the catabolism of cholesterol. They modulate bile flow and lipid secretion, are essential for the absorption of dietary fats and vitamins, and have been implicated in the regulation of all the key enzymes involved in cholesterol homeostasis. Bile acids recirculate through the liver, bile ducts, small intestine, and portal vein to form an enterohepatic circuit. They exist as anions at physiological pH, and consequently require a carrier for transport across the membranes of the enterohepatic tissues. The unique detergent properties of bile acids are essential for the digestion and intestinal absorption of hydrophobic nutrients. Bile acids have potent toxic properties (e.g. membrane disruption) and there are a plethora of mechanisms to limit their accumulation in blood and tissues (PMID: 11316487, 16037564, 12576301, 11907135). When present in sufficiently high levels, lithocholic acid can act as an oncometabolite. An oncometabolite is a compound that when present at chronically high levels promotes tumour growth and survival. Chronically high levels of lithocholic acid are associated with several forms of cancer including colon cancer, pancreatic cancer, esophageal cancer, and many other GI cancers. High bile acid levels lead to the generation of reactive oxygen species and reactive nitrogen species, disruption of the cell membrane and mitochondria, induction of DNA damage, mutation and apoptosis, and the development of reduced apoptosis capability upon chronic exposure (PMID: 24884764). Dietary fibre can bind to lithocholic acid and aid in its excretion in stool. As such, fibre can protect against colon cancer.
Metabolic

PW_C000609

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5-Hydroxyindoleacetic acid

5-hydroxyindoleacetic acid (5HIAA) is a breakdown product of serotonin that is excreted in the urine. Serotonin is a hormone found at high levels in many body tissues. Serotonin and 5HIAA are produced in excess amounts by carcinoid tumors, and levels of these substances may be measured in the urine to test for carcinoid tumors. (NCI).
Metabolic

PW_C000627

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Sapropterin

Sapropterin, also known as THBP or 6R-BH4, belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. They are mainly synthesized in several parts of the body, including the pineal gland. Sapropterin is slightly soluble (in water) and a very weakly acidic compound (based on its pKa). Sapropterin has been detected in multiple biofluids, such as blood and cerebrospinal fluid. Within the cell, sapropterin is primarily located in the cytoplasm. In humans, sapropterin is involved in catecholamine biosynthesis pathway. Sapropterin is also involved in a couple of metabolic disorders, which include aromatic L-aminoacid decarboxylase deficiency and tyrosine hydroxylase deficiency. Sapropterin is converted from 7,8-dihydroneopterin triphosphate by 6-pyruvoyl tetrahydropterin synthase and sepiapterin reductase. It is essential in the formation of neurotransmitters and for nitric oxide synthase (PMID 16946131).
Metabolic

PW_C000635

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Pristanic acid

Pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) is a terpenoid acid present at micromolar concentrations in the plasma of healthy individuals. It is also found in the lipids from many sources such as freshwater sponges, krill, earthworms, whales, human milk fat, bovine depot fat, butterfat or Californian petroleum. It is usually present in combination with phytanic acid. In humans, pristanic acid is obtained from two sources: either directly from the diet or as the alpha oxidation product of phytanic acid. At physiological concentrations pristanic acid is a natural ligand for PPARalpha. In liver, pristanic acid is degraded by peroxisomal beta oxidation to propionyl-CoA. Together with phytanic acid, pristanic acid accumulates in several inherited disorders such as Zellweger syndrome. Pristanic acid is a branched chain fatty acid that arises from the breakdown of phytanic acid. It is present at micromolar concentrations in the plasma of healthy individuals. Pristanic acid is normally degraded by peroxisomal beta-oxidation. In patients affected with generalized peroxisomal disorders, degradation of both phytanic acid and pristanic acid is impaired owing to absence of functional peroxisomes. Pristanic acid has been found to activate the peroxisome proliferator-activated receptor {alpha} (PPAR{alpha}) in a concentration dependent manner.
Metabolic

PW_C000637

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SAICAR

SAICAR (or (S)-2-[5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido]succinate) is a substrate for the multifunctional protein ADE2. SAICAR is an intermediate in purine metabolism. SAICAR is converted from 5-amino-1-(5-phospho-D-ribosyl) imidazole-4-carboxylate via phosphoribosylaminoimidazole-succinocarboxamide synthase (EC: 6.3.2.6) or SAICAR synthase. This enzyme catalyses the seventh step out of ten in the biosynthesis of purine nucleotides. The appearance of succinylaminoimidazolecarboxamide riboside (SAICAriboside) and succinyladenosine (S-Ado) in cerebrospinal fluid, in urine, and, to a lesser extent, in plasma is characteristic of a heritable deficiency known as adenylosuccinate lyase deficiency. Adenylosuccinate lyase deficiency is responsible for a range of symptoms that involve psychomotor retardation, often accompanied by epileptic seizures, and autistic features. When present in sufficiently high levels, SAICAR can act as an oncometabolite, a metabotoxin, and an acidogen. An oncometabolite is a compound that promotes tumour growth and survival. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. As an oncometabolite, high levels of SAICAR stimulate pyruvate kinase isoform M2 and promote cancer cell survival in glucose-limited conditions such as aerobic glycolysis (PMID: 23086999). As an organic acid, SAICAR is associated with acidosis. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated adenylosuccinate lyase deficiency. Many affected children with organic acidemias experience intellectual disability or delayed development.
Metabolic

PW_C000639

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Phytanic acid

Phytanic acid (or 3,7,11,15-tetramethylhexadecanoic acid) is a 20-carbon branched-chain fatty acid that humans can obtain through the consumption of dairy products, ruminant animal fats, and certain fish. It is primarily formed by bacterial degradation of chlorophyll in the intestinal tract of ruminants. Unlike most fatty acids, phytanic acid cannot be metabolized by beta-oxidation (because of a methyl group in the beta position). Instead, it undergoes alpha-oxidation in the peroxisome, where it is converted into pristanic acid by the removal of one carbon. Pristanic acid can undergo several rounds of beta-oxidation in the peroxisome to form medium-chain fatty acids that can be converted into carbon dioxide and water in mitochondria. Refsum disease, an autosomal recessive neurological disorder caused by mutations in the PHYH gene, is characterized by having impaired alpha-oxidation activity. Individuals with Refsum disease accumulate large stores of phytanic acid in their blood and tissues. This frequently leads to peripheral polyneuropathy, cerebellar ataxia, retinitis pigmentosa, anosmia, and hearing loss. Therefore, chronically high levels of phytanic acid can be neurotoxic. Phytanic acid's neurotoxicity appears to lie in its ability to initiate astrocyte/neural cell death by activating the mitochondrial route of apoptosis. In particular, phytanic acid can induce the substantial generation of reactive oxygen species in isolated mitochondria as well as in intact cells. It also induces the release of cytochrome c from mitochondria.
Metabolic

PW_C000643

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Myristic acid

Myristic acid is a saturated 14-carbon fatty acid occurring in most animal and vegetable fats, particularly butterfat and coconut, palm, and nutmeg oils. It is used to synthesize flavor and as an ingredient in soaps and cosmetics. (From Dorland, 28th ed). Myristic acid is also commonly added to a penultimate nitrogen terminus glycine in receptor-associated kinases to confer the membrane localisation of the enzyme. this is achieved by the myristic acid having a high enough hydrophobicity to become incorporated into the fatty acyl core of the phospholipid bilayer of the plasma membrane of the eukaryotic cell.(wikipedia).
Drug Metabolism
Drug Action

PW_C000644

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3-Phosphoglyceric acid

3-phosphoglyceric acid (3PG) is a 3-carbon molecule that is a metabolic intermediate in both glycolysis and the Calvin cycle. This chemical is often termed PGA when referring to the Calvin cycle. In the Calvin cycle, two glycerate 3-phosphate molecules are reduced to form two molecules of glyceraldehyde 3-phosphate (GALP). (wikipedia).
Metabolic

PW_C000649

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N-Acetyl-L-aspartic acid

N-Acetylaspartic acid is a derivative of aspartic acid. It is the second most concentrated molecule in the brain after the amino acid glutamate. It is synthesized in neurons from the amino acid aspartate and acetyl coenzyme A (acetyl CoA). The various functions served by N-acetylaspartic acid are still under investigation, but the primary proposed functions include (1) acting as a neuronal osmolyte that is involved in fluid balance in the brain, (2) serving as a source of acetate for lipid and myelin synthesis in oligodendrocytes (the glial cells that myelinate neuronal axons), (3) serving as a precursor for the synthesis of the important dipeptide neurotransmitter N-acetylaspartylglutamate (NAAG), and (4) playing a potential role in energy production from the amino acid glutamate in neuronal mitochondria. High neurotransmitter levels can lead to abnormal neural signaling, delayed or arrested intellectual development, and difficulties with general motor skills. When present in sufficiently high levels, N-acetylaspartic acid can be a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural tissue. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of N-acetylaspartic acid are associated with Canavan disease. N-acetylaspartic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. Infants with acidosis have symptoms that include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, seizures, coma, and possibly death. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and flapping tremors.
Metabolic

PW_C000654

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Normetanephrine

Normetanephrine, also known as normetadrenaline, belongs to the class of organic compounds known as methoxyphenols. Methoxyphenols are compounds containing a methoxy group attached to the benzene ring of a phenol moiety. Normetanephrine exists as a solid, slightly soluble (in water), and a very weakly acidic compound (based on its pKa). Normetanephrine has been found in human brain and endocrine gland tissues, and has also been primarily detected in feces, urine, blood, and cerebrospinal fluid. Within the cell, normetanephrine is primarily located in the cytoplasm. In humans, normetanephrine is involved in the tyrosine metabolism pathway and the disulfiram action pathway. Normetanephrine is also involved in several metabolic disorders, some of which include the tyrosinemia type I pathway, tyrosinemia, transient, OF the newborn pathway, dopamine beta-hydroxylase deficiency, and the alkaptonuria pathway. Normetanephrine is a methylated metabolite of norepinephrine that is excreted in the urine and found in certain tissues. It is a marker for tumors.
Metabolic

PW_C000655

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Propyl alcohol

Propyl alcohol is a colorless liquid made by oxidation of aliphatic hydrocarbons that is used as a solvent and chemical intermediate. Propyl alcohol is a primary alcohol with the formula CH3CH2CH2OH. It is also known as propan-1-ol, 1-propanol, 1-propyl alcohol, n-propyl alcohol, or simply propanol. It is an isomer of propan-2-ol. It is used as a solvent in the pharmaceutical industry, and for resins and cellulose esters. It is formed naturally in small amounts during many fermentation processes and small amounts are produced by gut microflora.
Drug Metabolism
Drug Action

PW_C000658

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Propionylcarnitine

Propionylcarnitine is present in high abundance in the urine of patients with Methylmalonyl-CoA mutase (MUT) deficiency, (together with Methylmalonic acid). MUT is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA (OMIM 609058 ).
Metabolic

PW_C000661

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Stearic acid

Stearic acid, also called octadecanoic acid, is one of the useful types of saturated fatty acids that comes from many animal and vegetable fats and oils. It is a waxy solid, and its chemical formula is CH3(CH2)16COOH. Its name comes from the Greek word stear, which means tallow. Its IUPAC name is octadecanoic acid. -- Wikipedia.
Drug Metabolism
Drug Action

PW_C000662

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Ureidosuccinic acid

Ureidosuccinic acid, also known as L-ureidosuccinate or carbamyl-L-aspartate, belongs to the class of organic compounds known as aspartic acid and derivatives. Aspartic acid and derivatives are compounds containing an aspartic acid or a derivative thereof resulting from reaction of aspartic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. Ureidosuccinic acid exists as a solid, soluble (in water), and a weakly acidic compound (based on its pKa). Ureidosuccinic acid has been found in human prostate tissue, and has also been primarily detected in saliva. Within the cell, ureidosuccinic acid is primarily located in the cytoplasm. Ureidosuccinic acid exists in all living organisms, ranging from bacteria to humans. In humans, ureidosuccinic acid is involved in the aspartate metabolism pathway and the pyrimidine metabolism pathway. Ureidosuccinic acid is also involved in several metabolic disorders, some of which include the canavan disease pathway, UMP synthase deficiency (orotic aciduria), dihydropyrimidinase deficiency, and Beta ureidopropionase deficiency. Outside of the human body, ureidosuccinic acid can be found in a number of food items such as green vegetables, celeriac, pepper (c. frutescens), and italian sweet red pepper. This makes ureidosuccinic acid a potential biomarker for the consumption of these food products. Ureidosuccinic acid is an intermediary product in pyrimidine biosynthesis.
Drug Metabolism
Drug Action

PW_C000673

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Neopterin

Neopterin is a pteridine derivative present in body fluids; elevated levels result from immune system activation, malignant disease, allograft rejection, and viral infections. (From Stedman, 26th ed) Neopterin also serves as a precursor in the biosynthesis of biopterin.
Drug Metabolism
Drug Action

PW_C000675

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Stearoylcarnitine

Stearoylcarnitine, also known as acylcarnitine C18:0, is a fatty ester lipid molecule. It is found in significantly greater amounts of patients with carnitine palmitoyltransferase (CPT) II deficiency when compared to controls (PMID: 15653102). Stearoylcarnitine is also found to be associated with celiac disease, another inborn error of metabolism. The carnitine palmitoyltransferase (CPT; EC 2.3.1.21) enzyme system, in conjunction with acyl-CoA synthetase and carnitine/acylcarnitine translocase, provides the mechanism whereby long-chain fatty acids are transferred from the cytosol to the mitochondrial matrix to undergo beta-oxidation (OMIM: 600650 ). Stearoylcarnitine is considered to be practically insoluble (in water) and acidic.
Metabolic

PW_C000680

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Formiminoglutamic acid

Formiminoglutamic acid, also known as N-formimino-L-glutamate or acid, formiminoglutamic, belongs to the class of organic compounds known as glutamic acid and derivatives. Glutamic acid and derivatives are compounds containing glutamic acid or a derivative thereof resulting from reaction of glutamic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. Formiminoglutamic acid is slightly soluble (in water) and a moderately acidic compound (based on its pKa). Formiminoglutamic acid has been detected in multiple biofluids, such as feces and urine. Within the cell, formiminoglutamic acid is primarily located in the cytoplasm. In humans, formiminoglutamic acid is involved in the histidine metabolism pathway. Formiminoglutamic acid is also involved in the metabolic disorder called the histidinemia pathway. Measurement of this acid in the urine after oral administration of histidine provides the basis for the diagnostic test of folic acid deficiency and of megaloblastic anemia of pregnancy.
Metabolic

PW_C000681

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Nicotinamide riboside

Nicotinamide riboside is involved in nicotinate and nicotinamide metabolism. Nicotinamide riboside was originally identified as a nutrient in milk. It is a useful compound for elevation of NAD+ levels in humans. Nicotinamide riboside has recently been discovered to be an NAD(+) precursor that is converted to nicotinamide mononucleotide by specific nicotinamide riboside kinases, Nrk1 and Nrk2. It has been shown that exogenous nicotinamide riboside promotes Sir2-dependent repression of recombination, improves gene silencing, and extends the lifespan of certain animal models without calorie restriction [PMID:17482543]. Supplementation in mammalian cells and mouse tissues increases NAD(+) levels and activates SIRT1 and SIRT3, culminating in enhanced oxidative metabolism and protection against high-fat diet-induced metabolic abnormalities. (PMID:22682224). Recent data suggest that nicotinamide riboside may be the only vitamin precursor that supports neuronal NAD+ synthesis (PMID: 18429699). Nicotinamide riboside kinase has an essential role for phosphorylation of nicotinamide riboside and the cancer drug tiazofurin (PMID 15137942).
Metabolic

PW_C000691

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Xylulose 5-phosphate

Xylulose 5-phosphate (Xu-5-P) is a metabolite of the hexose monophosphate pathway that activates protein phosphatase 2A to mediate the acute effects of carbohydrate feeding on the glycolytic pathway, as well as the coordinate long-term control of the enzymes required for fatty acid and triglyceride synthesis. Xu-5-P is the signal for the coordinated control of lipogenesis. Feeding carbohydrate causes levels of liver glucose, Glucose-6-phosphate (Glc-6-P), and Fructose-6-phosphate (Fru-6-P) to rise. Elevation of Fru-6-P leads to elevation of Xu-5-P in reactions catalyzed by the near-equilibrium isomerases of the nonoxidative portion of the hexose monophosphate pathway (ribulose 5-phosphate (Ru5P) epimerase [EC 5.1.3.1], ribose 5-phosphate (Rib5P) isomerase [EC 5.3.1.6], transaldolase [EC 2.2.1.2], and transketolase [EC 2.2.1.1]). The elevation of Xu-5-P is the coordinating signal that both acutely activates phosphofructokinase [PFK; EC 2.7.1.11] in glycolysis and promotes the action of the transcription factor carbohydrate responsive element binding protein (ChREBP) to increase transcription of the genes for the enzymes of lipogenesis, the hexose monophosphate shunt, and glycolysis, all of which are required for the de novo synthesis of fat. (PMID 12721358).
Drug Metabolism
Drug Action
Showing 261 - 280 of 55735 compounds