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L-2-hydroxyglutarate Dehydrogenase
In enzymology, an L-2-hydroxyglutarate dehydrogenase () is an enzyme that catalyzes the chemical reaction :(S)-2-hydroxyglutarate + acceptor \rightleftharpoons 2-oxoglutarate + reduced acceptor Thus, the two substrates of this enzyme are (S)-2-hydroxyglutarate and acceptor, whereas its two products are 2-oxoglutarate and reduced acceptor. Enzymes which preferentially catalyze the conversion of the (R) stereoisomer of 2-oxoglutarate also exist in both mammals and plants and are named D-2-hydroxyglutarate dehydrogenase. L-2-hydroxyglutarate is produced by promiscuous action of malate dehydrogenase on 2-oxoglutarate; L-2-hydroxyglutarate dehydrogenase is an example of a metabolite repair enzyme that oxidizes L-2-hydroxyglutarate back to 2-oxoglutarate. Nomenclature This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with other acceptors. The systematic name of this enzyme class is (S)-2-hydroxyglutarate:acceptor ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Enzymology
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. Almost all metabolic processes in the cell need enzyme catalysis in order to occur at rates fast enough to sustain life. Metabolic pathways depend upon enzymes to catalyze individual steps. The study of enzymes is called ''enzymology'' and the field of pseudoenzyme analysis recognizes that during evolution, some enzymes have lost the ability to carry out biological catalysis, which is often reflected in their amino acid sequences and unusual 'pseudocatalytic' properties. Enzymes are known to catalyze more than 5,000 biochemical reaction types. Other biocatalysts are catalytic RNA molecules, called ribozymes. Enzymes' specificity comes from their unique three-dimensional structures. Like all catalysts, enzymes increase the reaction ra ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Metabolite Damage And Its Repair Or Pre-emption
Metabolite damage can occur through enzyme promiscuity or spontaneous chemical reactions. Many metabolites are Chemical reaction, chemically reactive and unstable and can react with other cell components or undergo unwanted modifications. Enzymatically or chemically damaged metabolites are always useless and often toxic. To prevent toxicity that can occur from the accumulation of damaged metabolites, organisms have damage-control systems that: # Reconvert damaged metabolites to their original, undamaged form (damage repair) # Convert a potentially harmful metabolite to a benign one (damage pre-emption) # Prevent damage from happening by limiting the build-up of reactive, but non-damaged metabolites that can lead to harmful products (directed overflow) Damage-control systems can involve one or more specific enzymes. Types of damage Similarly to DNA and proteins, metabolites are prone to damage, which can occur chemically or through enzyme promiscuity. Much less is known about metabo ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hydroxyacid-oxoacid Transhydrogenase
In enzymology, a hydroxyacid-oxoacid transhydrogenase () is an enzyme that catalyzes the chemical reaction :(S)-3-hydroxybutanoate + 2-oxoglutarate \rightleftharpoons acetoacetate + (R)-2-hydroxyglutarate Thus, the two substrates of this enzyme are (S)-3-hydroxybutanoate and 2-oxoglutarate, whereas its two products are acetoacetate and (R)-2-hydroxyglutarate. This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with other acceptors. The systematic name of this enzyme class is (S)-3-hydroxybutanoate:2-oxoglutarate oxidoreductase. This enzyme is also called transhydrogenase, hydroxy acid-oxo acid. See also * D2HGDH * L2HGDH * 2-hydroxyglutarate synthase * 2-hydroxyglutarate dehydrogenase * Alpha-Hydroxyglutaric acid * 2-Hydroxyglutaric aciduria 2-hydroxyglutaric aciduria is a rare neurometabolic disorder characterized by the significantly elevated levels of hydroxyglutaric acid in one's urine. It is either autosom ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Alpha-Hydroxyglutaric Acid
α-Hydroxyglutaric acid (2-hydroxyglutaric acid) is an alpha hydroxy acid form of glutaric acid. In biology In humans the compound is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria is formed by a 2-hydroxyglutarate synthase. The compound can be converted to α-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase which, in humans, are two enzymes called D2HGDH and L2HGDH. Deficiency in either of these two enzymes lead to a disease known as 2-hydroxyglutaric aciduria. D-2-hydroxyglutarate Mutations in isocitrate dehydrogenase (IDH1 and IDH2), which frequently occur in glioma and AML, produce D-2-hydroxyglutarate from alpha-ketoglutarate. D-2-hydroxyglutarate accumulates to very high concentrations which inhibits the function of enzymes that are dependent on alpha-ketoglutarate, including histone lysine demethylases. This leads to a hypermethylated state of DNA and histones, which results in different gene expression that can activa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
2-hydroxyglutarate Synthase
In enzymology, a 2-hydroxyglutarate synthase () is an enzyme that catalyzes the chemical reaction :propanoyl-CoA + H2O + glyoxylate \rightleftharpoons 2-hydroxyglutarate + CoA The 3 substrates of this enzyme are propanoyl-CoA, H2O, and glyoxylate, whereas its two products are 2-hydroxyglutarate and CoA. This enzyme belongs to the family of transferases, specifically those acyltransferases that convert acyl groups into alkyl groups on transfer. The systematic name of this enzyme class is propanoyl-CoA:glyoxylate C-propanoyltransferase (thioester-hydrolysing, 2-carboxyethyl-forming). Other names in common use include 2-hydroxyglutaratic synthetase, 2-hydroxyglutaric synthetase, alpha-hydroxyglutarate synthase, hydroxyglutarate synthase, and 2-hydroxyglutarate glyoxylate-lyase (CoA-propanoylating). This enzyme participates in c5-branched dibasic acid metabolism. See also * D2HGDH * L2HGDH * 2-hydroxyglutarate dehydrogenase * 2-Hydroxyglutaric aciduria * Hydroxyacid-oxoacid ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
D2HGDH
D-2-hydroxyglutarate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the ''D2HGDH'' gene. This gene encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. See also * L2HGDH * 2-hydroxyglutarate synthase * 2-hydroxyglutarate dehydrogenase * Hydroxyacid-oxoacid transhydrogenase In enzymology, a hydroxyacid-oxoacid transhydrogenase () is an enzyme that catalyzes the chemical reaction :(S)-3-hydroxybutanoate + 2-oxoglutarate \rightleftharpoons acetoacetate + (R)-2-hydroxyglutarate Thus, the two substrates of this enzym ... References Further reading< ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
2-Hydroxyglutaric Aciduria
2-hydroxyglutaric aciduria is a rare neurometabolic disorder characterized by the significantly elevated levels of hydroxyglutaric acid in one's urine. It is either autosomal recessive or autosomal dominant. Presentation The signs/symptoms of this condition are consistent with the following: *Intellectual disability, *Muscular hypotonia *Encephalitis *Seizures *Aphasia Cause Mutation in several genes can lead to different types of 2-hydroxyglutaric aciduria. For example, the D2HGDH and L2HGDH genes provide instructions for making enzymes that are found in mitochondria - in which these enzymes break down D-2-hydroxyglutarate and L-2-hydroxyglutarate, respectively, as a part of normal reaction series that generate energy for cell activities. Any mutations occur in either of these genes would interrupt the functional enzymes and allow both 2-hydroxyglutarates to accumulate in cells, which cause 2-hydroxyglutaric aciduria type I. Moreover, it is known that type II for L-2-hydroxygl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Arabidopsis Thaliana
''Arabidopsis thaliana'', the thale cress, mouse-ear cress or arabidopsis, is a small flowering plant native to Eurasia and Africa. ''A. thaliana'' is considered a weed; it is found along the shoulders of roads and in disturbed land. A winter annual with a relatively short lifecycle, ''A. thaliana'' is a popular model organism in plant biology and genetics. For a complex multicellular eukaryote, ''A. thaliana'' has a relatively small genome around 135 mega base pairs. It was the first plant to have its genome sequenced, and is a popular tool for understanding the molecular biology of many plant traits, including flower development and light sensing. Description ''Arabidopsis thaliana'' is an annual (rarely biennial) plant, usually growing to 20–25 cm tall. The leaves form a rosette at the base of the plant, with a few leaves also on the flowering stem. The basal leaves are green to slightly purplish in color, 1.5–5 cm long, and 2–10 mm broad, with an ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
L2HGDH
L-2-hydroxyglutarate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the ''L2HGDH'' gene, also known as C14orf160, on chromosome 14. Function This gene encodes L-2-hydroxyglutarate dehydrogenase, a flavin adenine dinucleotide (FAD)-dependent enzyme that oxidizes Alpha-Hydroxyglutaric acid, L-2-hydroxyglutarate to alpha-ketoglutarate in a variety of mammalian tissues. Mutations in this gene cause L-2-hydroxyglutaric aciduria, a rare autosomal recessive neurometabolic disorder resulting in moderate to severe mental retardation. L2HGDH codes for a protein that is 50 kDa in size. The L2HGDH protein contains a mitochondrial-targeting transit peptide and is localized to the mitochondrial inner membrane inside mitochondria inside the cell. The L2HGDH protein catalyzes the following reaction, and requires flavin adenine dinucleotide (FAD) as a Cofactor (biochemistry), co-factor: (S)-2-hydroxyglutarate + acceptor = 2-oxoglutarate + reduced acceptor. L-2-hydrox ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
List Of Enzymes
This article lists enzymes by their classification in the International Union of Biochemistry and Molecular Biology's Enzyme Commission (EC) numbering system. * List of EC numbers (EC 5) * List of EC numbers (EC 6) :Oxidoreductases (EC 1) (Oxidoreductase) *Dehydrogenase * Luciferase *DMSO reductase :EC 1.1 (act on the CH-OH group of donors) * :EC 1.1.1 (with NAD+ or NADP+ as acceptor) ** Alcohol dehydrogenase (NAD) ** Alcohol dehydrogenase (NADP) **Homoserine dehydrogenase ** Aminopropanol oxidoreductase **Diacetyl reductase **Glycerol dehydrogenase **Propanediol-phosphate dehydrogenase ** glycerol-3-phosphate dehydrogenase (NAD+) ** D-xylulose reductase **L-xylulose reductase **Lactate dehydrogenase **Malate dehydrogenase **Isocitrate dehydrogenase ** HMG-CoA reductase * :EC 1.1.2 (with a cytochrome as acceptor) * :EC 1.1.3 (with oxygen as acceptor) **Glucose oxidase **L-gulonolactone oxidase **Thiamine oxidase **Xanthine oxidase * :EC 1.1.4 (with a disul ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Oxidoreductase
In biochemistry, an oxidoreductase is an enzyme that catalyzes the transfer of electrons from one molecule, the reductant, also called the electron donor, to another, the oxidant, also called the electron acceptor. This group of enzymes usually utilizes NADP+ or NAD+ as cofactors. Transmembrane oxidoreductases create electron transport chains in bacteria, chloroplasts and mitochondria, including respiratory complexes I, II and III. Some others can associate with biological membranes as peripheral membrane proteins or be anchored to the membranes through a single transmembrane helix. in ...
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Malate Dehydrogenase
Malate dehydrogenase () (MDH) is an enzyme that reversibly catalyzes the oxidation of malate to oxaloacetate using the reduction of NAD+ to NADH. This reaction is part of many metabolic pathways, including the citric acid cycle. Other malate dehydrogenases, which have other EC numbers and catalyze other reactions oxidizing malate, have qualified names like malate dehydrogenase (NADP+). Isozymes Several isozymes of malate dehydrogenase exist. There are two main isoforms in eukaryotic cells. One is found in the mitochondrial matrix, participating as a key enzyme in the citric acid cycle that catalyzes the oxidation of malate. The other is found in the cytoplasm, assisting the malate-aspartate shuttle with exchanging reducing equivalents so that malate can pass through the mitochondrial membrane to be transformed into oxaloacetate for further cellular processes. Humans and most other mammals express the following two malate dehydrogenases: Protein families The m ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
