Gene Association: HMGN2
UniProt Search:
HMGN2 (PROTEIN_CODING)
Function Description: high mobility group nucleosomal binding domain 2
found 9 associated metabolites with current gene based on the text mining result from the pubmed database.
3-hydroxy-3-methylglutarate
3-Hydroxymethylglutaric acid is an "off-product" intermediate in the leucine degradation process. It is produced by defective or inefficient versions of 3-hydroxy-3-methylglutaryl-CoA lyase, an enzyme that normally catalyzes the conversion of 3-hydroxy-3-methylglutaryl-CoA to acetyl-CoA and acetoacetate. If this enzyme is defective, 3-hydroxy-3-methylglutaryl-CoA will accumulate in the mitochondria. Increased concentrations of 3-hydroxy-3-methylglutaryl-CoA can lead to a disruption of the esterified CoA:free CoA ratio and ultimately to mitochondrial toxicity. Detoxification of these CoA end products occurs via the transfer of the 3-hydroxymethylglutaryl moiety to carnitine, forming 3-hydroxymethylglutaric-carnitine, which is then transferred across the inner mitochondrial membrane where 3-hydroxymethylglutaric acid is released as the free acid. 3-Hydroxymethylglutaric acid has been found to accumulate in the urine of patients affected by 3-Hydroxy-3-methylglutaric aciduria, a rare inborn error of metabolism (OMIM: 246450). 3-Hydroxy-3-methylglutaric aciduria is caused by significantly reduced enzyme activity of the intramitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase (EC 4.1.3.4), the enzyme that catalyzes the final step of leucine degradation. This enzyme also plays a key role in ketone body formation. The profile of urinary organic acids for individuals with 3-hydroxy-3-methylglutaric aciduria is different from that of the other identified defects of leucine degradation, such as maple syrup urine disease (OMIM: 248600), isovaleric acidemia (OMIM: 243500), and methylcrotonylglycinemia (OMIM: 210200). The urinary organic acid profile of 3-hydroxy-3-methylglutaric aciduria includes elevated concentrations of 3-hydroxy-3-isovaleric, 3-hydroxy-3-methylglutaric, 3-methylglutaconic, and 3-methylglutaric acids (PMID: 10916782, 9658458, 3063529). Clinical manifestations of 3-hydroxy-3-methylglutaric aciduria include hepatomegaly, lethargy, coma, and apnea. Biochemically, there is a characteristic absence of ketosis with hypoglycemia, acidosis, hypertransaminasemia, and variable hyperammonemia. Therefore, when present in sufficiently high concentrations, 3-hydroxymethylglutaric acid can act as an acidogen and a metabotoxin. 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 noted above, chronically high levels of 3-hydroxymethylglutaric acid are associated with the inborn error of metabolism 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. 3-Hydroxymethylglutaric 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. 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 the untreated IEMs mentioned above. 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 seizures. 3-hydroxymethylglutaric acid, also known as meglutol or dicrotalic acid, is a member of the class of compounds known as hydroxy fatty acids. Hydroxy fatty acids are fatty acids in which the chain bears a hydroxyl group. 3-hydroxymethylglutaric acid is soluble (in water) and a weakly acidic compound (based on its pKa). 3-hydroxymethylglutaric acid can be synthesized from glutaric acid. 3-hydroxymethylglutaric acid is also a parent compound for other transformation products, including but not limited to, viscumneoside VII, viscumneoside IV, and yanuthone D. 3-hydroxymethylglutaric acid can be found in flaxseed, which makes 3-hydroxymethylglutaric acid a potential biomarker for the consumption of this food product. 3-hydroxymethylglutaric acid can be found primarily in saliva and urine. 3-hydroxymethylglutaric acid is a non-carcinogenic (not listed by IARC) potentially toxic compound. Chronically high levels of 3-hydroxymethylglutaric acid are associated with the inborn error of metabolism: 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency (T3DB). Meglutol is an antilipidemic agent that lowers cholesterol, triglycerides, and serum beta-lipoproteins and phospholipids, and inhibits hydroxymethylglutaryl-CoA reductase activity, which is the rate-limiting enzyme in cholesterol biosynthesis. Meglutol is an antilipidemic agent that lowers cholesterol, triglycerides, and serum beta-lipoproteins and phospholipids, and inhibits hydroxymethylglutaryl-CoA reductase activity, which is the rate-limiting enzyme in cholesterol biosynthesis.
Cinchonidine
Cinchonine is found in fruits. Cinchonine is an alkaloid from the leaves of Olea europaea Cinchonine is an alkaloidwith molecular formula C19H22N2O used in asymmetric synthesis in organic chemistry. It is a stereoisomer and pseudo-enantiomer of cinchonidine D000890 - Anti-Infective Agents > D000977 - Antiparasitic Agents > D000981 - Antiprotozoal Agents [Raw Data] CB216_Cinchonine_pos_10eV_CB000075.txt [Raw Data] CB216_Cinchonine_pos_30eV_CB000075.txt [Raw Data] CB216_Cinchonine_pos_40eV_CB000075.txt [Raw Data] CB216_Cinchonine_pos_50eV_CB000075.txt [Raw Data] CB216_Cinchonine_pos_20eV_CB000075.txt Alkaloid from the leaves of Olea europaea Cinchonidine (α-Quinidine) is a cinchona alkaloid found in Cinchona officinalis and Gongronema latifolium. A building block used in asymmetric synthesis in organic chemistry. Weak inhibitor of serotonin transporter (SERT) with Kis of 330, 4.2, 36, 196, 15 μM for dSERT, hSERT, hSERT I172M, hSERT S438T, hSERT Y95F, respectively. Antimalarial activities[1]. Cinchonidine (α-Quinidine) is a cinchona alkaloid found in Cinchona officinalis and Gongronema latifolium. A building block used in asymmetric synthesis in organic chemistry. Weak inhibitor of serotonin transporter (SERT) with Kis of 330, 4.2, 36, 196, 15 μM for dSERT, hSERT, hSERT I172M, hSERT S438T, hSERT Y95F, respectively. Antimalarial activities[1]. Cinchonine is a natural compound present in Cinchona bark. Cinchonine activates endoplasmic reticulum stress-induced apoptosis in human liver cancer cells[1]. Cinchonine is a natural compound present in Cinchona bark. Cinchonine activates endoplasmic reticulum stress-induced apoptosis in human liver cancer cells[1].
Pyocyanin
An iminium betaine that is 5-methylphenazin-5-ium which is substituted at position 1 by an oxidanidyl group. An antibiotic pigment produced by Pseudomonas aeruginosa. CONFIDENCE standard compound; INTERNAL_ID 184 Pyocyanin (Pyocyanine) is a phenazine that is a toxic, quorum sensing (QS)-controlled metabolite produced by P. aeruginosa. Pyocyanin is a redox-active compound and promotes the generation of reactive oxygen species (ROS). Pyocyanin also possesses antibacterial properties and increases fitness in competition with other bacterial species[1].
3-Hydroxy-3-methylglutaric acid
D057847 - Lipid Regulating Agents > D000960 - Hypolipidemic Agents > D000924 - Anticholesteremic Agents C - Cardiovascular system > C10 - Lipid modifying agents > C10A - Lipid modifying agents, plain D004791 - Enzyme Inhibitors > D019161 - Hydroxymethylglutaryl-CoA Reductase Inhibitors C78276 - Agent Affecting Digestive System or Metabolism > C29703 - Antilipidemic Agent D009676 - Noxae > D000963 - Antimetabolites Meglutol is an antilipidemic agent that lowers cholesterol, triglycerides, and serum beta-lipoproteins and phospholipids, and inhibits hydroxymethylglutaryl-CoA reductase activity, which is the rate-limiting enzyme in cholesterol biosynthesis. Meglutol is an antilipidemic agent that lowers cholesterol, triglycerides, and serum beta-lipoproteins and phospholipids, and inhibits hydroxymethylglutaryl-CoA reductase activity, which is the rate-limiting enzyme in cholesterol biosynthesis.
Sanazin
Pyocyanin (Pyocyanine) is a phenazine that is a toxic, quorum sensing (QS)-controlled metabolite produced by P. aeruginosa. Pyocyanin is a redox-active compound and promotes the generation of reactive oxygen species (ROS). Pyocyanin also possesses antibacterial properties and increases fitness in competition with other bacterial species[1].
EDTA disodium salt
D064449 - Sequestering Agents > D002614 - Chelating Agents > D065096 - Calcium Chelating Agents C78275 - Agent Affecting Blood or Body Fluid > C263 - Anticoagulant Agent D000074385 - Food Ingredients > D005503 - Food Additives D006401 - Hematologic Agents > D000925 - Anticoagulants
EDTA disodium
D064449 - Sequestering Agents > D002614 - Chelating Agents > D065096 - Calcium Chelating Agents C78275 - Agent Affecting Blood or Body Fluid > C263 - Anticoagulant Agent D000074385 - Food Ingredients > D005503 - Food Additives D006401 - Hematologic Agents > D000925 - Anticoagulants
