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Succinate
Succinic acid () is a dicarboxylic acid with the chemical formula (CH2)2(CO2H)2. In living organisms, succinic acid takes the form of an anion, succinate, which has multiple biological roles as a metabolic intermediate being converted into Fumaric acid, fumarate by the enzyme succinate dehydrogenase in complex 2 of the electron transport chain which is involved in making Adenosine triphosphate, ATP, and as a signaling molecule reflecting the cellular metabolic state. Succinate is generated in mitochondria via the citric acid cycle, tricarboxylic acid (TCA) cycle. Succinate can exit the mitochondrial matrix and function in the cytoplasm as well as the extracellular space, changing gene expression patterns, modulating epigenetic landscape or demonstrating hormone-like signaling. As such, succinate links cellular metabolism, especially ATP formation, to the regulation of cellular function. Dysregulation of succinate synthesis, and therefore ATP synthesis, happens in some genetic m ...
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Succinate Dehydrogenase
Succinate dehydrogenase (SDH) or succinate-coenzyme Q reductase (SQR) or respiratory complex II is an enzyme complex, found in many bacterial cells and in the inner mitochondrial membrane of eukaryotes. It is the only enzyme that participates in both the citric acid cycle and oxidative phosphorylation. Histochemical analysis showing high succinate dehydrogenase in muscle demonstrates high mitochondrial content and high oxidative potential. In step 6 of the citric acid cycle, SQR catalyzes the oxidation of succinate to fumarate with the reduction of ubiquinone to ubiquinol. This occurs in the inner mitochondrial membrane by coupling the two reactions together. Structure Subunits Mitochondrial and many bacterial SQRs are composed of four structurally different subunits: two hydrophilic and two hydrophobic. The first two subunits, a flavoprotein (SDHA) and an iron-sulfur protein (SDHB), form a hydrophilic head where enzymatic activity of the complex takes place. SD ...
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Citric Acid Cycle
The citric acid cycle—also known as the Krebs cycle, Szent–Györgyi–Krebs cycle, or TCA cycle (tricarboxylic acid cycle)—is a series of chemical reaction, biochemical reactions that release the energy stored in nutrients through acetyl-CoA Redox, oxidation. The energy released is available in the form of Adenosine triphosphate, ATP. The Hans Krebs (biochemist), Krebs cycle is used by organisms that generate energy via Cellular respiration, respiration, either anaerobic respiration, anaerobically or aerobic respiration, aerobically (organisms that Fermentation, ferment use different pathways). In addition, the cycle provides precursor (chemistry), precursors of certain amino acids, as well as the reducing agent nicotinamide adenine dinucleotide, NADH, which are used in other reactions. Its central importance to many Metabolic pathway, biochemical pathways suggests that it was one of the earliest metabolism components. Even though it is branded as a "cycle", it is not necessa ...
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Electron Transport Chain
An electron transport chain (ETC) is a series of protein complexes and other molecules which transfer electrons from electron donors to electron acceptors via redox reactions (both reduction and oxidation occurring simultaneously) and couples this electron transfer with the transfer of protons (H+ ions) across a membrane. Many of the enzymes in the electron transport chain are embedded within the membrane. The flow of electrons through the electron transport chain is an exergonic process. The energy from the redox reactions creates an electrochemical proton gradient that drives the synthesis of adenosine triphosphate (ATP). In aerobic respiration, the flow of electrons terminates with molecular oxygen as the final electron acceptor. In anaerobic respiration, other electron acceptors are used, such as sulfate. In an electron transport chain, the redox reactions are driven by the difference in the Gibbs free energy of reactants and products. The free energy released when ...
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Melas Syndrome
MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes) is one of the family of mitochondrial diseases, which also include MIDD (maternally inherited diabetes and deafness), MERRF syndrome, and Leber's hereditary optic neuropathy. It was first characterized under this name in 1984. A feature of these diseases is that they are caused by defects in the mitochondrial genome which is inherited purely from the female parent. The most common MELAS mutation is one in mitochondrial DNA (mtDNA) referred to as m.3243A>G. Signs and symptoms MELAS is a condition that affects many of the body's systems, particularly the brain and nervous system (encephalo-) and muscles (myopathy). As such, it is commonly referred to as a mitochondrial encephalomyopathy, due to the co-occurrence of these pathologies. In most cases, the signs and symptoms of this disorder appear in childhood following a period of normal development. Children with MELAS often have normal early psych ...
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Leigh Syndrome
Leigh syndrome (also called Leigh disease and subacute necrotizing encephalomyelopathy) is an inherited neurometabolic disorder that affects the central nervous system. It is named after Archibald Denis Leigh, a British neuropsychiatrist who first described the condition in 1951. Normal levels of thiamine, thiamine monophosphate, and thiamine diphosphate are commonly found, but there is a reduced or absent level of thiamine triphosphate. This is thought to be caused by a blockage in the enzyme thiamine-diphosphate kinase, and therefore treatment in some patients would be to take thiamine triphosphate daily. While the majority of patients typically exhibit symptoms between the ages of 3 and 12 months, instances of adult onset have also been documented. Signs and symptoms The symptoms of Leigh syndrome were classically described as beginning in infancy and leading to death within a span of several years; however, as more cases are recognized, it is apparent that symptoms can em ...
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Mitochondria
A mitochondrion () is an organelle found in the cells of most eukaryotes, such as animals, plants and fungi. Mitochondria have a double membrane structure and use aerobic respiration to generate adenosine triphosphate (ATP), which is used throughout the cell as a source of chemical energy. They were discovered by Albert von Kölliker in 1857 in the voluntary muscles of insects. The term ''mitochondrion'', meaning a thread-like granule, was coined by Carl Benda in 1898. The mitochondrion is popularly nicknamed the "powerhouse of the cell", a phrase popularized by Philip Siekevitz in a 1957 ''Scientific American'' article of the same name. Some cells in some multicellular organisms lack mitochondria (for example, mature mammalian red blood cells). The multicellular animal '' Henneguya salminicola'' is known to have retained mitochondrion-related organelles despite a complete loss of their mitochondrial genome. A large number of unicellular organisms, such as microspo ...
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Aqueous Solution
An aqueous solution is a solution in which the solvent is water. It is mostly shown in chemical equations by appending (aq) to the relevant chemical formula. For example, a solution of table salt, also known as sodium chloride (NaCl), in water would be represented as . The word ''aqueous'' (which comes from ''aqua'') means pertaining to, related to, similar to, or dissolved in, water. As water is an excellent solvent and is also naturally abundant, it is a ubiquitous solvent in chemistry. Since water is frequently used as the solvent in experiments, the word solution refers to an aqueous solution, unless the solvent is specified. A ''non-aqueous solution'' is a solution in which the solvent is a liquid, but is not water. Characteristics Substances that are ''hydrophobic'' ('water-fearing') do not dissolve well in water, whereas those that are '' hydrophilic'' ('water-friendly') do. An example of a hydrophilic substance is sodium chloride. In an aqueous solution the hydrogen ...
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Amber
Amber is fossilized tree resin. Examples of it have been appreciated for its color and natural beauty since the Neolithic times, and worked as a gemstone since antiquity."Amber" (2004). In Maxine N. Lurie and Marc Mappen (eds.) ''Encyclopedia of New Jersey'', Rutgers University Press, . Amber is used in jewelry and as a healing agent in Traditional medicine, folk medicine. There are five classes of amber, defined on the basis of their chemical constituents. Because it originates as a soft, sticky tree resin, amber sometimes contains animal and plant material as Inclusion (mineral), inclusions. Amber occurring in coal seams is also called resinite, and the term ''ambrite'' is applied to that found specifically within New Zealand coal seams. Etymology The English word ''amber'' derives from Arabic from Middle Persian 𐭠𐭭𐭡𐭫 (''ʾnbl'' /⁠ambar⁠/, “ambergris”) via Medieval Latin, Middle Latin ''ambar'' and Middle French ''ambre''. The word referred to what is n ...
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E-numbers
E numbers, short for Europe numbers, are codes for substances used as food additives, including those found naturally in many foods, such as vitamin C, for use within the European Union (EU) and European Free Trade Association (EFTA). Commonly found on food labels, their safety assessment and approval are the responsibility of the European Food Safety Authority (EFSA). The fact that an additive has an E number implies that its use was at one time permitted in products for sale in the European Single Market; some of these additives are no longer allowed today. Having a single unified list for food additives was first agreed upon in 1962 with food colouring. In 1964, the directives for preservatives were added, in 1970 antioxidants were added, in 1974 emulsifiers, stabilisers, thickeners and gelling agents were added as well. Numbering schemes The numbering scheme follows that of the International Numbering System (INS) as determined by the ''Codex Alimentarius'' committee, th ...
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Inflammation
Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', ''dolor'', ''rubor'', ''tumor'', and ''functio laesa''). Inflammation is a generic response, and therefore is considered a mechanism of innate immunity, whereas adaptive immunity is specific to each pathogen. Inflammation is a protective response involving immune cells, blood vessels, and molecular mediators. The function of inflammation is to eliminate the initial cause of cell injury, clear out damaged cells and tissues, and initiate tissue repair. Too little inflammation could lead to progressive tissue destruction by the harmful stimulus (e.g. bacteria) and compromise the survival of the organism. However inflammation can also have negative effects. Too much inflammation, in the form of chronic inflammation, is associated with variou ...
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Malignancy
Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not self-limited in its growth, is capable of invading into adjacent tissues, and may be capable of spreading to distant tissues. A benign tumor has none of those properties, but may still be harmful to health. The term benign in more general medical use characterizes a condition or growth that is not cancerous, i.e. does not spread to other parts of the body or invade nearby tissue. Sometimes the term is used to suggest that a condition is not dangerous or serious. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis. Malignant tumors are also characterized by genome instability, so that cancers, as assessed by whole genome sequencing, frequently have between 10,000 and 100,000 mutations in their entire genomes. ...
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