PW_C000001
HMDB0000001:
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1-Methylhistidine
One-methylhistidine (1-MHis) is derived mainly from the anserine of dietary flesh sources, especially poultry. The enzyme, carnosinase, splits anserine into b-alanine and 1-MHis. High levels of 1-MHis tend to inhibit the enzyme carnosinase and increase anserine levels. Conversely, genetic variants with deficient carnosinase activity in plasma show increased 1-MHis excretions when they consume a high meat diet. Reduced serum carnosinase activity is also found in patients with Parkinson's disease and multiple sclerosis and patients following a cerebrovascular accident. Vitamin E deficiency can lead to 1-methylhistidinuria from increased oxidative effects in skeletal muscle.
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PW_C000002
HMDB0000002:
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1,3-Diaminopropane
1,3-Diaminopropane is a stable, flammable and highly hydroscopic fluid. It is a polyamine that is normally quite toxic if swallowed, inhaled or absorbed through the skin. It is a catabolic byproduct of spermidine. It is also a precursor in the enzymatic synthesis of beta-alanine. 1, 3-Diaminopropane is involved in the arginine/proline metabolic pathways and the beta-alanine metabolic pathway.
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PW_C000003
HMDB0000005:
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2-Ketobutyric acid
2-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. More specifically, alpha-ketobutyric acid is a product of the lysis of cystathionine. It is also one of the degradation products of threonine. It can be converted into propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted into succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle.
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PW_C000004
HMDB0000008:
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2-Hydroxybutyric acid
2-Hydroxybutyric acid (alpha-hydroxybutyrate) is an organic acid derived from alpha-ketobutyrate. alpha-Ketobutyrate is produced by amino acid catabolism (threonine and methionine) and glutathione anabolism (cysteine formation pathway) and is metabolized to propionyl-CoA and carbon dioxide (PMID: 20526369). 2-Hydroxybutyric acid is formed as a by-product of the formation of alpha-ketobutyrate via a reaction catalyzed by lactate dehydrogenase (LDH) or alpha-hydroxybutyrate dehydrogenase (alphaHBDH). alpha-Hydroxybutyric acid is primarily produced in mammalian hepatic tissues that catabolize L-threonine or synthesize glutathione. Oxidative stress or detoxification of xenobiotics in the liver can dramatically increase the rate of hepatic glutathione synthesis. Under such metabolic stress conditions, supplies of L-cysteine for glutathione synthesis become limiting, so homocysteine is diverted from the transmethylation pathway (which forms methionine) into the transsulfuration pathway (which forms cystathionine). 2-Hydroxybutyrate is released as a byproduct when cystathionine is cleaved into cysteine that is incorporated into glutathione. Chronic shifts in the rate of glutathione synthesis may be reflected by urinary excretion of 2-hydroxybutyrate. 2-Hydroxybutyrate is an early marker for both insulin resistance and impaired glucose regulation that appears to arise due to increased lipid oxidation and oxidative stress (PMID: 20526369). 2-Hydroxybutyric acid is often found in the urine of patients suffering from lactic acidosis and ketoacidosis. 2-Hydroxybutyric acid generally appears at high concentrations in situations related to deficient energy metabolism (e.g. birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. More recently it has been noted that elevated levels of alpha-hydroxybutyrate in the plasma is a good marker for early-stage type II diabetes (PMID: 19166731). It was concluded from studies done in the mid-1970's that an increased NADH2/NAD ratio was the most important factor for the production of 2-hydroxybutyric acid (PMID: 168632).
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PW_C000005
HMDB0000010:
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2-Methoxyestrone
2-Methoxyestrone is a steroid derivative that is a byproduct of estrone and 2-hydroxyestrone metabolism. It is part of the androgen and estrogen metabolic pathway. The acid ionization constant (pKa) of 2-methoxyestrone is 10.81 (PMID: 516114). 2-Methoxyestrone can be metabolized to a sulfated derivative (2-methoxyestrone 3-sulfate) via steroid sulfotransferase (EC 2.8.2.15). It can also be glucuronidated to 2-methoxyestrone 3-glucuronide by UDP glucuronosyltransferase (EC 2.4.1.17).
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PW_C000006
HMDB0000011:
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(R)-3-Hydroxybutyric acid
(R)-3-Hydroxybutyric acid is a butyric acid substituted with a hydroxyl group in the beta or 3 position. 3-hydroxybutyric acid, or beta-hydroxybutyrate, is involved in the synthesis and degradation of ketone bodies. Like the other ketone bodies (acetoacetate and acetone), levels of beta-hydroxybutyrate are raised in the blood and urine in ketosis. Beta-hydroxybutyrate is a typical partial-degradation product of branched-chain amino acids (primarily valine) released from muscle for hepatic and renal gluconeogenesis This acid is metabolized by 3-hydroxybutyrate dehydrogenase (catalyzes the oxidation of D-3-hydroxybutyrate to form acetoacetate, using NAD+ as an electron acceptor). The enzyme functions in nervous tissues and muscles, enabling the use of circulating hydroxybutyrate as a fuel. In the liver mitochondrial matrix, the enzyme can also catalyze the reverse reaction, a step in ketogenesis. 3-Hydroxybutyric acid is a chiral compound having two enantiomers, D-3-hydroxybutyric acid and L-3-hydroxybutyric acid. In humans, beta-hydroxybutyrate is synthesized in the liver from acetyl-CoA, and can be used as an energy source by the brain when blood glucose is low. It can also be used for the synthesis of biodegradable plastics (Wikipedia).
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PW_C000007
HMDB0000012:
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Deoxyuridine
2'-Deoxyuridine is a naturally occurring nucleoside. It is similar in chemical structure to uridine, but without the 2'-hydroxyl group. It is considered to be an antimetabolite that is converted to deoxyuridine triphosphate during DNA synthesis. Laboratory suppression of deoxyuridine is used to diagnose megaloblastic anemia due to vitamin B12 and folate deficiencies.
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PW_C000008
HMDB0000014:
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Deoxycytidine
One of the principal nucleosides of DNA composed of cytosine and deoxyribose. A nucleoside consists of only a pentose sugar linked to a purine or pyrimidine base, without a phosphate group. When N1 is linked to the C1 of deoxyribose, deoxynucleosides and nucleotides are formed from cytosine and deoxyribose; deoxycytidine monophosphate (dCMP), deoxycytidine diphosphate (dCDP), deoxycytidine triphosphate (dCTP). CTP is the source of the cytidine in RNA (ribonucleic acid) and deoxycytidine triphosphate (dCTP) is the source of the deoxycytidine in DNA (deoxyribonucleic acid).
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PW_C000009
HMDB0000015:
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Cortexolone
Cortexolone is the precursor of cortisol. Accumulation of cortexolone can happen in a defect known as congenital adrenal hyperplasia, which is due to 11-beta-hydroxylase deficiency, resulting in androgen excess, virilization, and hypertension (PMID: 2022736). Cortexolone is a 17-hydroxycorticosteroid with glucocorticoid and anti-inflammatory activities (PubChem).
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PW_C000010
HMDB0000016:
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Deoxycorticosterone
11-Deoxycorticosterone (also called desoxycortone, 21-hydroxyprogesterone, DOC, or simply deoxycorticosterone) is a steroid hormone produced by the adrenal gland that possesses mineralocorticoid activity and acts as a precursor to aldosterone. It is classified as a member of the 21-hydroxysteroids. 21-hydroxysteroids are steroids carrying a hydroxyl group at the 21-position of the steroid backbone. Deoxycorticosterone is very hydrophobic, practically insoluble (in water), and relatively neutral. Deoxycorticosterone can be synthesized from progesterone by 21-beta-hydroxylase and is then converted to corticosterone by 11-beta-hydroxylase. Corticosterone is then converted to aldosterone by aldosterone synthase. Deoxycorticosterone stimulates the collecting tubules in the kidney to continue to excrete potassium in much the same way that aldosterone does. Deoxycorticosterone has about 1/20 of the sodium retaining power of aldosterone and about 1/5 the potassium excreting power of aldosterone (Wikipedia). Deoxycorticosterone can be found throughout all human tissues and has been detected in amniotic fluid and blood. When present in sufficiently high levels, deoxycorticosterone can act as a hypertensive agent and a metabotoxin. A hypertensive agent increases blood pressure and causes the production of more urine. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of deoxycorticosterone are associated with congenital adrenal hyperplasia (CAH) and with adrenal tumors producing deoxycorticosterone (PMID: 20671982). High levels of this mineralocorticoid are associated with resistant hypertension, which can result in polyuria, polydipsia, increased blood volume, edema, and cardiac enlargement. Deoxycorticosterone can be used to treat adrenal insufficiency. In particular, desoxycorticosterone acetate (DOCA) is used as replacement therapy in Addison's disease.
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PW_C000011
HMDB0000017:
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4-Pyridoxic acid
4-Pyridoxic acid is the catabolic product of vitamin B6 (also known as pyridoxine, pyridoxal and pyradoxamine) which is excreted in the urine. Urinary levels of 4-pyridoxic acid are lower in females than in males and will be reduced in persons with riboflavin deficiency. 4-Pyridoxic acid is formed by the action of aldehyde oxidase I (an endogenous enzyme) and by microbial enzymes (pyridoxal 4-dehydrogenase), an NAD-dependent aldehyde dehydrogenase. 4-pyridoxic acid can be further broken down by the gut microflora via 4-pyridoxic acid dehydrogenase. This enzyme catalyzes the four electron oxidation of 4-pyridoxic acid to 3-hydroxy-2-methylpyridine-4,5-dicarboxylate, using nicotinamide adenine dinucleotide as a cofactor.
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PW_C000012
HMDB0000019:
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α-Ketoisovaleric acid
alpha-Ketoisovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. alpha-Ketoisovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. 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 alpha-ketoisovaleric acid are associated with maple syrup urine disease. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465). alpha-Ketoisovaleric acid is a keto-acid, which is a subclass of organic acids. 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 MSUD. Many affected children with organic acidemias experience intellectual disability or delayed development.
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PW_C000013
HMDB0000020:
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p-Hydroxyphenylacetic acid
p-Hydroxyphenylacetic acid is an oxidative deaminated metabolite of p-tyramine. Also a metabolite of tyrosine via enteric bacteria. The bacterial origin of this compound was confirmed by the finding that this compound in urine decreased significantly after the use of the antibiotic neomycin.
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PW_C000014
HMDB0000021:
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Iodotyrosine
Iodotyrosine is an iodated derivative of L-tyrosine. This is an early precursor to L-thyroxine, one of the primary thyroid hormones. In the thyroid gland, iodide is trapped, transported, and concentrated in the follicular lumen for thyroid hormone synthesis. Before trapped iodide can react with tyrosine residues, it must be oxidized by thyroid peroxidase. Iodotyrosine is made from tyrosine via thyroid peroxidase and then further iodinated by this enzyme to make the di-iodo and tri-iodo variants. Two molecules of di-iodotyrosine combine to form T4, and one molecule of mono-iodotyrosine combines with one molecule of di-iodotyrosine to form T3.
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PW_C000015
HMDB0000022:
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3-Methoxytyramine
The O-methylated derivative of dopamine. Dopamine is methylated by catechol-O-methyltransferase (COMT) to make 3-Methoxytyramine. This compound can be broken down to homovanillic acid by monoamine oxidase and aldehyde dehydrogenase. Elevated concentrations of this compound are indicated for a variety of brain and carcinoid tumors as well as certain mental disorders.
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PW_C000016
HMDB0000023:
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(S)-3-Hydroxyisobutyric acid
(S)-3-Hydroxyisobutyric (3-HIBA) acid is an organic acid. 3-HIBA is an intermediate in L-valine metabolism. 3-HIBA plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (OMIM 236795) and methylmalonic semialdehyde dehydrogenase deficiency (OMIM 603178). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. 3-hydroxyisobutyric aciduria is caused by a 3-hydroxyisobutyryl-CoA dehydrogenase deficiency (PMID: 18329219). The severity of this disease varies from case to case. Most patients exhibit dysmorphic features, such as a small triangular face, a long philtrum, low set ears and micrognathia (PMID: 113770040, 10686279). Lactic acidemia is also found in the affected patients, indicating that mitochondrial dysfunction is involved. 3-hydroxyisobutyrate appears to specifically inhibit the function of the respiratory chain complex I-III and mitochondrial creatine kinase (PMID: 18329219).
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PW_C000017
HMDB0000024:
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3-O-Sulfogalactosylceramide (d18:1/24:0)
3-O-Sulfogalactosylceramide is an acidic, sulfated glycosphingolipid, often known as sulfatide. This lipid occurs in membranes of various cell types, but is found in particularly high concentrations in myelin where it constitutes 3-4% of total membrane lipids. This lipid is synthesized primarily in the oligodendrocytes in the central nervous system. Accumulation of this lipid in the lysosomes is a characteristic of metachromatic leukodystrophy, a lysosomal storage disease caused by the deficiency of arylsulfatase A. Alterations in sulfatide metabolism, trafficking, and homeostasis are present in the earliest clinically recognizable stages of Alzheimer's disease. Cerebrosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Cerebrosides have a single sugar group linked to ceramide. The most common are galactocerebrosides (containing galactose), the least common are glucocerebrosides (containing glucose). Galactocerebrosides are found predominantly in neuronal cell membranes. In contrast glucocerebrosides are not normally found in membranes. Instead, they are typically intermediates in the synthesis or degradation of more complex glycosphingolipids. Galactocerebrosides are synthesized from ceramide and UDP-galactose. Excess lysosomal accumulation of glucocerebrosides is found in Gaucher disease. Sulfatides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Sulfatides are the sulfuric acid esters of galactocerebrosides. They are synthesized from galactocerebrosides and activated sulfate, 3'-phosphoadenosine 5'-phosphosulfate (PAPS).
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PW_C000018
HMDB0000026:
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Ureidopropionic acid
Ureidopropionic acid is an intermediate in the metabolism of uracil. More specifically it is a breakdown product of dihydrouracil and is produced by the enzyme dihydropyrimidase. It is further decomposed to beta-alanine via the enzyme beta-ureidopropionase. Ureidopropionic acid is essentially a urea derivative of beta-alanine. High levels of Ureidopropionic acid are found in individuals with beta-ureidopropionase (UP) deficiency [PMID: 11675655]. Enzyme deficiencies in pyrimidine metabolism are associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil.
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PW_C000019
HMDB0000027:
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Tetrahydrobiopterin
Tetrahydrobiopterin or BH4 is a cofactor in the synthesis of nitric oxide. In fact it is used by all three human nitric-oxide synthases (NOS) eNOS, nNOS, and iNOS as well as the enzyme glyceryl-ether monooxygenase. It is also essential in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine to L-dopa by the enzyme tyrosine hydroxylase; and conversion of tryptophan to 5-hydroxytryptophan via tryptophan hydroxylase. Specifically, tetrahydrobiopterin is a cofactor for tryptophan 5-hydroxylase 1, tyrosine 3-monooxygenase, and phenylalanine hydroxylase all of which are essential for the formation of the neurotransmitters dopamine, noradrenaline and adrenaline. Tetrahydrobiopterin has been proposed to be involved in promotion of neurotransmitter release in the brain and the regulation of human melanogenesis. A defect in BH4 production and/or a defect in the enzyme dihydropteridine reductase (DHPR) causes phenylketonuria type IV, as well as dopa-responsive dystonias. BH4 is also implicated in Parkinson's disease, Alzheimer's disease and depression. Tetrahydrobiopterin is present in probably every cell or tissue of higher animals. On the other hand, most bacteria, fungi and plants do not synthesize tetrahydrobiopterin. -- Wikipedia.
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PW_C000020
HMDB0000030:
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Biotin
Biotin is an enzyme co-factor present in minute amounts in every living cell. Biotin is also known as vitamin H or B7 or coenzyme R. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. Biotin has been recognized as an essential nutrient. Our biotin requirement is fulfilled in part through diet, through endogenous reutilization of biotin and perhaps through capture of biotin generated in the intestinal flora. The utilization of biotin for covalent attachment to carboxylases and its reutilization through the release of carboxylase biotin after proteolytic degradation constitutes the 'biotin cycle'. Biotin deficiency is associated with neurological manifestations, skin rash, hair loss and metabolic disturbances that are thought to relate to the various carboxylase deficiencies (metabolic ketoacidosis with lactic acidosis). It has also been suggested that biotin deficiency is associated with protein malnutrition, and that marginal biotin deficiency in pregnant women may be teratogenic. Biotin acts as a carboxyl carrier in carboxylation reactions. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lysine residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lys residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. Evidence is emerging that biotin participates in processes other than classical carboxylation reactions. Specifically, novel roles for biotin in cell signaling, gene expression, and chromatin structure have been identified in recent years. Human cells accumulate biotin by using both the sodium-dependent multivitamin transporter and monocarboxylate transporter 1. These transporters and other biotin-binding proteins partition biotin to compartments involved in biotin signaling: cytoplasm, mitochondria, and nuclei. The activity of cell signals such as biotinyl-AMP, Sp1 and Sp3, nuclear factor (NF)-kappaB, and receptor tyrosine kinases depends on biotin supply. Consistent with a role for biotin and its catabolites in modulating these cell signals, greater than 2000 biotin-dependent genes have been identified in various human tissues. Many biotin-dependent gene products play roles in signal transduction and localize to the cell nucleus, consistent with a role for biotin in cell signaling. Posttranscriptional events related to ribosomal activity and protein folding may further contribute to effects of biotin on gene expression. Finally, research has shown that biotinidase and holocarboxylase synthetase mediate covalent binding of biotin to histones (DNA-binding proteins), affecting chromatin structure; at least seven biotinylation sites have been identified in human histones. Biotinylation of histones appears to play a role in cell proliferation, gene silencing, and the cellular response to DNA repair. Roles for biotin in cell signaling and chromatin structure are consistent with the notion that biotin has a unique significance in cell biology. (PMID: 15992684, 16011464).
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- 2-Hydroxyglutric Aciduria (D and L Form) (Homo sapiens)
- 2-Hydroxyglutric Aciduria (D and L Form) (Mus musculus)
- 2-Hydroxyglutric Aciduria (D and L Form) (Rattus norvegicus)
- 2-Ketoglutarate Dehydrogenase Complex Deficiency (Homo sapiens)
- 2-Ketoglutarate Dehydrogenase Complex Deficiency (Mus musculus)
- 2-Ketoglutarate Dehydrogenase Complex Deficiency (Rattus norvegicus)
- 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency (Mus musculus)
- 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency (Rattus norvegicus)
- 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency (Homo sapiens)
- 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency (Homo sapiens)
- 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency (Mus musculus)
- 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency (Rattus norvegicus)
- 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency (Homo sapiens)
- 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency (Mus musculus)
- 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency (Rattus norvegicus)
- 3-Hydroxyisobutyric Aciduria (Homo sapiens)
- 3-Hydroxyisobutyric Aciduria (Mus musculus)
- 3-Hydroxyisobutyric Aciduria (Rattus norvegicus)
- 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I (Homo sapiens)
- 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I (Mus musculus)
- 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I (Rattus norvegicus)
- 3-Methylglutaconic Aciduria Type I (Homo sapiens)
- 3-Methylglutaconic Aciduria Type I (Mus musculus)
- 3-Methylglutaconic Aciduria Type I (Rattus norvegicus)
- 3-Methylglutaconic Aciduria Type III (Homo sapiens)
- 3-Methylglutaconic Aciduria Type III (Mus musculus)
- 3-Methylglutaconic Aciduria Type III (Rattus norvegicus)
- 3-Methylglutaconic Aciduria Type IV (Homo sapiens)
- 3-Methylglutaconic Aciduria Type IV (Mus musculus)
- 3-Methylglutaconic Aciduria Type IV (Rattus norvegicus)
- 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency (Homo sapiens)
- 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency (Mus musculus)
- 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency (Rattus norvegicus)
- Aerobic Glycolysis (Warburg Effect) (Homo sapiens)
- Alanine Metabolism (Homo sapiens)
- Alanine Metabolism (Mus musculus)
- Alanine Metabolism (Bos taurus)
- Alanine Metabolism (Rattus norvegicus)
- Alanine Metabolism (Drosophila melanogaster)
- Alanine Metabolism (Caenorhabditis elegans)
- Ammonia Recycling (Homo sapiens)
- Ammonia Recycling (Mus musculus)
- Ammonia Recycling (Bos taurus)
- Ammonia Recycling (Rattus norvegicus)
- Ammonia Recycling (Drosophila melanogaster)
- Ammonia Recycling (Caenorhabditis elegans)
- beta-Ketothiolase Deficiency (Homo sapiens)
- beta-Ketothiolase Deficiency (Mus musculus)
- beta-Ketothiolase Deficiency (Rattus norvegicus)
- Biotin Metabolism (Homo sapiens)
- Biotin Metabolism (Escherichia coli)
- Biotin Metabolism (Mus musculus)
- Biotin Metabolism (Bos taurus)
- Biotin Metabolism (Rattus norvegicus)
- Biotin Metabolism (Drosophila melanogaster)
- Biotin Metabolism (Pseudomonas aeruginosa)
- Biotin-Carboxyl Carrier Protein Assembly (Escherichia coli)
- Biotin-Carboxyl Carrier Protein Assembly (Pseudomonas aeruginosa)
- Biotinidase Deficiency (Homo sapiens)
- Biotinidase Deficiency (Mus musculus)
- Biotinidase Deficiency (Rattus norvegicus)
- Citric Acid Cycle (Homo sapiens)
- Citric Acid Cycle (Mus musculus)
- Citric Acid Cycle (Bos taurus)
- Citric Acid Cycle (Rattus norvegicus)
- Citric Acid Cycle (Drosophila melanogaster)
- Citric Acid Cycle (Caenorhabditis elegans)
- Congenital Lactic Acidosis (Homo sapiens)
- Congenital Lactic Acidosis (Mus musculus)
- Congenital Lactic Acidosis (Rattus norvegicus)
- Fatty Acid Biosynthesis (Homo sapiens)
- Fatty Acid Biosynthesis (Saccharomyces cerevisiae)
- Fatty Acid Biosynthesis (Mus musculus)
- Fatty Acid Biosynthesis (Bos taurus)
- Fatty Acid Biosynthesis (Rattus norvegicus)
- Fatty Acid Biosynthesis (Drosophila melanogaster)
- Fatty Acid Biosynthesis (Caenorhabditis elegans)
- Fructose-1,6-diphosphatase Deficiency (Homo sapiens)
- Fructose-1,6-diphosphatase Deficiency (Mus musculus)
- Fructose-1,6-diphosphatase Deficiency (Rattus norvegicus)
- Fumarase Deficiency (Homo sapiens)
- Fumarase Deficiency (Mus musculus)
- Fumarase Deficiency (Rattus norvegicus)
- Gluconeogenesis (Homo sapiens)
- Gluconeogenesis (Mus musculus)
- Gluconeogenesis (Bos taurus)
- Gluconeogenesis (Rattus norvegicus)
- Glutamate Metabolism (Homo sapiens)
- Glutamate Metabolism (Mus musculus)
- Glutamate Metabolism (Bos taurus)
- Glutamate Metabolism (Rattus norvegicus)
- Glutamate Metabolism (Drosophila melanogaster)
- Glutamate Metabolism (Caenorhabditis elegans)
- Glutaminolysis and Cancer (Homo sapiens)
- Glutaminolysis and Cancer (Mus musculus)
- Glutaminolysis and Cancer (Rattus norvegicus)
- Glycogen Storage Disease Type 1A (GSD1A) or Von Gierke Disease (Homo sapiens)
- Glycogen Storage Disease Type 1A (GSD1A) or Von Gierke Disease (Mus musculus)
- Glycogen Storage Disease Type 1A (GSD1A) or Von Gierke Disease (Rattus norvegicus)
- Glycogenosis, Type IA. Von Gierke Disease (Homo sapiens)
- Glycogenosis, Type IA. Von Gierke Disease (Mus musculus)
- Glycogenosis, Type IA. Von Gierke Disease (Rattus norvegicus)
- Glycogenosis, Type IB (Homo sapiens)
- Glycogenosis, Type IB (Mus musculus)
- Glycogenosis, Type IB (Rattus norvegicus)
- Glycogenosis, Type IC (Homo sapiens)
- Glycogenosis, Type IC (Mus musculus)
- Glycogenosis, Type IC (Rattus norvegicus)
- Homocarnosinosis (Homo sapiens)
- Homocarnosinosis (Mus musculus)
- Homocarnosinosis (Rattus norvegicus)
- Hyperinsulinism-Hyperammonemia Syndrome (Homo sapiens)
- Hyperinsulinism-Hyperammonemia Syndrome (Mus musculus)
- Hyperinsulinism-Hyperammonemia Syndrome (Rattus norvegicus)
- Isobutyryl-CoA Dehydrogenase Deficiency (Homo sapiens)
- Isobutyryl-CoA Dehydrogenase Deficiency (Mus musculus)
- Isobutyryl-CoA Dehydrogenase Deficiency (Rattus norvegicus)
- Isovaleric Acidemia (Homo sapiens)
- Isovaleric Acidemia (Mus musculus)
- Isovaleric Acidemia (Rattus norvegicus)
- Isovaleric Aciduria (Homo sapiens)
- Isovaleric Aciduria (Mus musculus)
- Isovaleric Aciduria (Rattus norvegicus)
- Lactic Acidemia (Homo sapiens)
- Lactic Acidemia (Mus musculus)
- Lactic Acidemia (Rattus norvegicus)
- Leigh Syndrome (Homo sapiens)
- Leigh Syndrome (Mus musculus)
- Leigh Syndrome (Rattus norvegicus)
- LPS and Citrate Signaling and Inflammation (Homo sapiens)
- LPS and Citrate Signaling and Inflammation (Mus musculus)
- LPS and Citrate Signaling and Inflammation (Bos taurus)
- LPS and Citrate Signaling and Inflammation (Rattus norvegicus)
- Malonic Aciduria (Mus musculus)
- Malonic Aciduria (Rattus norvegicus)
- Malonic Aciduria (Homo sapiens)
- Malonyl-CoA Decarboxylase Deficiency (Mus musculus)
- Malonyl-CoA Decarboxylase Deficiency (Rattus norvegicus)
- Malonyl-CoA Decarboxylase Deficiency (Homo sapiens)
- Maple Syrup Urine Disease (Homo sapiens)
- Maple Syrup Urine Disease (Mus musculus)
- Maple Syrup Urine Disease (Rattus norvegicus)
- Methylmalonate Semialdehyde Dehydrogenase Deficiency (Homo sapiens)
- Methylmalonate Semialdehyde Dehydrogenase Deficiency (Mus musculus)
- Methylmalonate Semialdehyde Dehydrogenase Deficiency (Rattus norvegicus)
- Methylmalonic Aciduria (Homo sapiens)
- Methylmalonic Aciduria (Mus musculus)
- Methylmalonic Aciduria (Rattus norvegicus)
- Methylmalonic Aciduria Due to Cobalamin-Related Disorders (Mus musculus)
- Methylmalonic Aciduria Due to Cobalamin-Related Disorders (Rattus norvegicus)
- Methylmalonic Aciduria Due to Cobalamin-Related Disorders (Homo sapiens)
- Mitochondrial Complex II Deficiency (Homo sapiens)
- Mitochondrial Complex II Deficiency (Mus musculus)
- Mitochondrial Complex II Deficiency (Rattus norvegicus)
- Multiple Carboxylase Deficiency, Neonatal or Early Onset Form (Homo sapiens)
- Multiple Carboxylase Deficiency, Neonatal or Early Onset Form (Mus musculus)
- Multiple Carboxylase Deficiency, Neonatal or Early Onset Form (Rattus norvegicus)
- Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1) (Homo sapiens)
- Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1) (Mus musculus)
- Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1) (Rattus norvegicus)
- Primary Hyperoxaluria II, PH2 (Homo sapiens)
- Primary Hyperoxaluria II, PH2 (Mus musculus)
- Primary Hyperoxaluria II, PH2 (Rattus norvegicus)
- Primary Hyperoxaluria Type I (Homo sapiens)
- Primary Hyperoxaluria Type I (Mus musculus)
- Primary Hyperoxaluria Type I (Rattus norvegicus)
- Propanoate Metabolism (Mus musculus)
- Propanoate Metabolism (Bos taurus)
- Propanoate Metabolism (Rattus norvegicus)
- Propanoate Metabolism (Drosophila melanogaster)
- Propanoate Metabolism (Caenorhabditis elegans)
- Propanoate Metabolism (Homo sapiens)
- Propionic Acidemia (Homo sapiens)
- Propionic Acidemia (Mus musculus)
- Propionic Acidemia (Rattus norvegicus)
- Pyruvate Carboxylase Deficiency (Homo sapiens)
- Pyruvate Carboxylase Deficiency (Mus musculus)
- Pyruvate Carboxylase Deficiency (Rattus norvegicus)
- Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency) (Homo sapiens)
- Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency) (Mus musculus)
- Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency) (Rattus norvegicus)
- Pyruvate Dehydrogenase Complex Deficiency (Homo sapiens)
- Pyruvate Dehydrogenase Complex Deficiency (Mus musculus)
- Pyruvate Dehydrogenase Complex Deficiency (Rattus norvegicus)
- Pyruvate Dehydrogenase Deficiency (E2) (Homo sapiens)
- Pyruvate Dehydrogenase Deficiency (E2) (Mus musculus)
- Pyruvate Dehydrogenase Deficiency (E2) (Rattus norvegicus)
- Pyruvate Dehydrogenase Deficiency (E3) (Homo sapiens)
- Pyruvate Dehydrogenase Deficiency (E3) (Mus musculus)
- Pyruvate Dehydrogenase Deficiency (E3) (Rattus norvegicus)
- Pyruvate Kinase Deficiency (Homo sapiens)
- Pyruvate Kinase Deficiency (Mus musculus)
- Pyruvate Kinase Deficiency (Rattus norvegicus)
- Pyruvate Metabolism (Homo sapiens)
- Pyruvate Metabolism (Mus musculus)
- Pyruvate Metabolism (Bos taurus)
- Pyruvate Metabolism (Rattus norvegicus)
- Pyruvate Metabolism (Drosophila melanogaster)
- Pyruvate Metabolism (Caenorhabditis elegans)
- Succinic Semialdehyde Dehydrogenase Deficiency (Homo sapiens)
- Succinic Semialdehyde Dehydrogenase Deficiency (Mus musculus)
- Succinic Semialdehyde Dehydrogenase Deficiency (Rattus norvegicus)
- TCA Cycle (Saccharomyces cerevisiae)
- The Oncogenic Action of 2-Hydroxyglutarate (Homo sapiens)
- The Oncogenic Action of 2-Hydroxyglutarate (Mus musculus)
- The Oncogenic Action of 2-Hydroxyglutarate (Rattus norvegicus)
- The Oncogenic Action of D-2-Hydroxyglutarate in Hydroxyglutaric aciduria (Homo sapiens)
- The Oncogenic Action of Fumarate (Homo sapiens)
- The Oncogenic Action of L-2-Hydroxyglutarate in Hydroxyglutaric aciduria (Homo sapiens)
- The Oncogenic Action of Succinate (Homo sapiens)
- Threonine and 2-Oxobutanoate Degradation (Homo sapiens)
- Threonine and 2-Oxobutanoate Degradation (Mus musculus)
- Threonine and 2-Oxobutanoate Degradation (Bos taurus)
- Threonine and 2-Oxobutanoate Degradation (Rattus norvegicus)
- Threonine and 2-Oxobutanoate Degradation (Drosophila melanogaster)
- Threonine and 2-Oxobutanoate Degradation (Caenorhabditis elegans)
- Transfer of Acetyl Groups into Mitochondria (Homo sapiens)
- Transfer of Acetyl Groups into Mitochondria (Mus musculus)
- Transfer of Acetyl Groups into Mitochondria (Bos taurus)
- Transfer of Acetyl Groups into Mitochondria (Rattus norvegicus)
- Transfer of Acetyl Groups into Mitochondria (Drosophila melanogaster)
- Transfer of Acetyl Groups into Mitochondria (Caenorhabditis elegans)
- Triosephosphate Isomerase Deficiency (Homo sapiens)
- Triosephosphate Isomerase Deficiency (Mus musculus)
- Triosephosphate Isomerase Deficiency (Rattus norvegicus)
- Valine, Leucine, and Isoleucine Degradation (Homo sapiens)
- Valine, Leucine, and Isoleucine Degradation (Mus musculus)
- Valine, Leucine, and Isoleucine Degradation (Bos taurus)
- Valine, Leucine, and Isoleucine Degradation (Rattus norvegicus)
- Warburg Effect (Mus musculus)
- Warburg Effect (Bos taurus)
- Warburg Effect (Rattus norvegicus)
- Warburg Effect (Drosophila melanogaster)
- Warburg Effect (Caenorhabditis elegans)
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