Methylglutaconyl-CoA Hydratase
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Methylglutaconyl-CoA Hydratase
3-Methylglutaconyl-CoA hydratase, also known as MG-CoA hydratase and AUH, is an enzyme () encoded by the ''AUH'' gene on chromosome 19. It is a member of the Enoyl-CoA hydratase, enoyl-CoA hydratase/isomerase superfamily, but it is the only member of that family that is able to bind to RNA. Not only does it bind to RNA, AUH has also been observed to be involved in the metabolic enzymatic activity, making it a dual-role protein. Mutations of this gene have been found to cause a disease called 3-Methylglutaconic Acuduria Type 1. Structure The enzyme AUH has a molecular mass of 32 kDa and the AUH gene consists of 18 exons, is 1.7 Base pair, kb long, and is mainly found in kidney, skeletal muscle, heart, liver, and spleen cells. AUH has a similar fold that is found in other members of the enoyl-CoA hydratase/isomerase family; however, it is a hexamer as a Dimer (chemistry), dimer of trimer (chemistry), trimers. Also unlike other members of its family, AUH's surface is positively char ...
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Enzyme
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 react ...
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Trimer (chemistry)
In chemistry, a trimer (; ) is a molecule or polyatomic anion formed by combination or association of three molecules or ions of the same substance. In technical jargon, a trimer is a kind of oligomer derived from three identical precursors often in competition with polymerization. Examples Alkyne trimerisation In 1866, Marcellin Berthelot reported the first example of cyclotrimerization, the conversion of acetylene to benzene. This process was commercialized: : Nitrile trimerization Symmetrical 1,3,5-triazines are prepared by trimerization of certain nitriles such as cyanogen chloride or cyanimide. Cyanogen chloride and cyanogen bromide each trimerize at elevated temperatures over a carbon catalyst. The chloride gives cyanuric chloride: : The bromide has an extended shelflife when refrigerated. Like the chloride, it undergoes ab exothermic trimerisation to form cyanuric bromide. This reaction is catalyzed by traces of bromine, metal salts, acids and bases. For th ...
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Nucleotide
Nucleotides are organic molecules consisting of a nucleoside and a phosphate. They serve as monomeric units of the nucleic acid polymers – deoxyribonucleic acid (DNA) and ribonucleic acid (RNA), both of which are essential biomolecules within all life-forms on Earth. Nucleotides are obtained in the diet and are also synthesized from common nutrients by the liver. Nucleotides are composed of three subunit molecules: a nucleobase, a five-carbon sugar ( ribose or deoxyribose), and a phosphate group consisting of one to three phosphates. The four nucleobases in DNA are guanine, adenine, cytosine and thymine; in RNA, uracil is used in place of thymine. Nucleotides also play a central role in metabolism at a fundamental, cellular level. They provide chemical energy—in the form of the nucleoside triphosphates, adenosine triphosphate (ATP), guanosine triphosphate (GTP), cytidine triphosphate (CTP) and uridine triphosphate (UTP)—throughout the cell for the ...
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AU-rich Element
Adenylate-uridylate-rich elements (AU-rich elements; AREs) are found in the 3' untranslated region (UTR) of many messenger RNAs (mRNAs) that code for proto-oncogenes, nuclear transcription factors, and cytokines. AREs are one of the most common determinants of RNA stability in mammalian cells. AREs are defined as a region with frequent adenine and uridine bases in a mRNA. They usually target the mRNA for rapid degradation. ARE-directed mRNA degradation is influenced by many exogenous factors, including phorbol esters, calcium ionophores, cytokines, and transcription inhibitors. These observations suggest that AREs play a critical role in the regulation of gene transcription during cell growth and differentiation, and the immune response. AREs have been divided into three classes with different sequences. The best characterised adenylate uridylate (AU)-rich Elements have a core sequence of AUUUA within U-rich sequences (for example WWWU(AUUUA)UUUW where W is A or U). This lies ...
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Organic Redox Reaction
Organic reductions or organic oxidations or organic redox reactions are redox reactions that take place with organic compounds. In organic chemistry oxidations and reductions are different from ordinary redox reactions, because many reactions carry the name but do not actually involve electron transfer.March Jerry; (1985). Advanced Organic Chemistry reactions, mechanisms and structure (3rd ed.). New York: John Wiley & Sons, inc. Instead the relevant criterion for organic oxidation is gain of oxygen and/or loss of hydrogen, respectively.''Organic Redox Systems: Synthesis, Properties, and Applications'', Tohru Nishinaga 2016 Simple functional groups can be arranged in order of increasing oxidation state. The oxidation numbers are only an approximation: When methane is oxidized to carbon dioxide its oxidation number changes from −4 to +4. Classical reductions include alkene reduction to alkanes and classical oxidations include oxidation of alcohols to aldehydes. In oxidati ...
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Mitochondrial Matrix
In the mitochondrion, the matrix is the space within the inner membrane. The word "matrix" stems from the fact that this space is viscous, compared to the relatively aqueous cytoplasm. The mitochondrial matrix contains the mitochondrial DNA, ribosomes, soluble enzymes, small organic molecules, nucleotide cofactors, and inorganic ions. /sup> The enzymes in the matrix facilitate reactions responsible for the production of ATP, such as the citric acid cycle, oxidative phosphorylation, oxidation of pyruvate, and the beta oxidation of fatty acids. The composition of the matrix based on its structures and contents produce an environment that allows the anabolic and catabolic pathways to proceed favorably for. The electron transport chain and enzymes in the matrix play a large role in the citric acid cycle and oxidative phosphorylation. The citric acid cycle produces NADH and FADH2 through oxidation that will be reduced in oxidative phosphorylation to produce ATP. The cytosolic, ...
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Catabolism
Catabolism () is the set of metabolic pathways that breaks down molecules into smaller units that are either oxidized to release energy or used in other anabolic reactions. Catabolism breaks down large molecules (such as polysaccharides, lipids, nucleic acids, and proteins) into smaller units (such as monosaccharides, fatty acids, nucleotides, and amino acids, respectively). Catabolism is the breaking-down aspect of metabolism, whereas anabolism is the building-up aspect. Cells use the monomers released from breaking down polymers to either construct new polymer molecules or degrade the monomers further to simple waste products, releasing energy. Cellular wastes include lactic acid, acetic acid, carbon dioxide, ammonia, and urea. The formation of these wastes is usually an oxidation process involving a release of chemical free energy, some of which is lost as heat, but the rest of which is used to drive the synthesis of adenosine triphosphate (ATP). This molecule ac ...
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Leucine
Leucine (symbol Leu or L) is an essential amino acid that is used in the biosynthesis of proteins. Leucine is an α-amino acid, meaning it contains an α- amino group (which is in the protonated −NH3+ form under biological conditions), an α- carboxylic acid group (which is in the deprotonated −COO− form under biological conditions), and a side chain isobutyl group, making it a non-polar aliphatic amino acid. It is essential in humans, meaning the body cannot synthesize it: it must be obtained from the diet. Human dietary sources are foods that contain protein, such as meats, dairy products, soy products, and beans and other legumes. It is encoded by the codons UUA, UUG, CUU, CUC, CUA, and CUG. Like valine and isoleucine, leucine is a branched-chain amino acid. The primary metabolic end products of leucine metabolism are acetyl-CoA and acetoacetate; consequently, it is one of the two exclusively ketogenic amino acids, with lysine being the other. It is the most import ...
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3-methylglutaconyl-CoA
3-Methylglutaconyl-CoA (MG-CoA), also known as β-methylglutaconyl-CoA, is an intermediate in the metabolism of leucine. It is metabolized into HMG-CoA. Leucine metabolism See also * Methylcrotonyl-CoA carboxylase * Methylglutaconyl-CoA hydratase 3-Methylglutaconyl-CoA hydratase, also known as MG-CoA hydratase and AUH, is an enzyme () encoded by the ''AUH'' gene on chromosome 19. It is a member of the Enoyl-CoA hydratase, enoyl-CoA hydratase/isomerase superfamily, but it is the only member ... References Organophosphates Thioesters of coenzyme A {{biochem-stub ...
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Catalyze
Catalysis () is the process of increasing the rate of a chemical reaction by adding a substance known as a catalyst (). Catalysts are not consumed in the reaction and remain unchanged after it. If the reaction is rapid and the catalyst recycles quickly, very small amounts of catalyst often suffice; mixing, surface area, and temperature are important factors in reaction rate. Catalysts generally react with one or more reactants to form intermediates that subsequently give the final reaction product, in the process of regenerating the catalyst. Catalysis may be classified as either homogeneous, whose components are dispersed in the same phase (usually gaseous or liquid) as the reactant, or heterogeneous, whose components are not in the same phase. Enzymes and other biocatalysts are often considered as a third category. Catalysis is ubiquitous in chemical industry of all kinds. Estimates are that 90% of all commercially produced chemical products involve catalysts at some stag ...
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Enoyl-CoA Hydratase
Enoyl-CoA hydratase (ECH) or crotonase is an enzyme that hydrates the double bond between the second and third carbons on 2-trans/cis-enoyl-CoA: ECH is essential to metabolizing fatty acids in beta oxidation to produce both acetyl CoA and energy in the form of ATP. ECH of rats is a hexameric protein (this trait is not universal, but human enzyme is also hexameric), which leads to the efficiency of this enzyme as it has 6 active sites. This enzyme has been discovered to be highly efficient, and allows people to metabolize fatty acids into energy very quickly. In fact this enzyme is so efficient that the rate for short chain fatty acids is equivalent to that of diffusion-controlled reactions. Metabolism Fatty acid metabolism ECH catalyzes the second step (hydratation) in the breakdown of fatty acids (β-oxidation). Fatty acid metabolism is how human bodies turn fats into energy. Fats in foods are generally in the form of triglycerols. These must be broken down in order ...
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Crystallographic
Crystallography is the experimental science of determining the arrangement of atoms in crystalline solids. Crystallography is a fundamental subject in the fields of materials science and solid-state physics ( condensed matter physics). The word "crystallography" is derived from the Greek word κρύσταλλος (''krystallos'') "clear ice, rock-crystal", with its meaning extending to all solids with some degree of transparency, and γράφειν (''graphein'') "to write". In July 2012, the United Nations recognised the importance of the science of crystallography by proclaiming that 2014 would be the International Year of Crystallography. denote a direction vector (in real space). * Coordinates in ''angle brackets'' or ''chevrons'' such as <100> denote a ''family'' of directions which are related by symmetry operations. In the cubic crystal system for example, would mean 00 10 01/nowiki> or the negative of any of those directions. * Miller indices in ''parenthese ...
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