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Electron-transferring-flavoprotein dehydrogenase

Index Electron-transferring-flavoprotein dehydrogenase

Electron-transferring-flavoprotein dehydrogenase (ETF dehydrogenase or electron transfer flavoprotein-ubiquinone oxidoreductase) is an enzyme that transfers electrons from electron-transferring flavoprotein in the mitochondrial matrix, to the ubiquinone pool in the inner mitochondrial membrane. [1]

29 relations: Acidosis, Acyl CoA dehydrogenase, Acyl-CoA, Amino acid, Carnitine, Coenzyme Q – cytochrome c reductase, Coenzyme Q10, Cysteine, Disproportionation, Electron transport chain, Electron-transferring flavoprotein, ETFDH, Fatty acid, Flavin adenine dinucleotide, Flavin group, Genetic disorder, Glutaric acidemia type 2, Hypoglycemia, Inner mitochondrial membrane, Iron–sulfur protein, Metabolism, Microbial metabolism, Mitochondrial matrix, Octanoyl-CoA, Oxidative phosphorylation, Riboflavin, Semiquinone, Spectrophotometry, Ubiquinol.

Acidosis

Acidosis is a process causing increased acidity in the blood and other body tissues (i.e., an increased hydrogen ion concentration).

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Acyl CoA dehydrogenase

Acyl-CoA dehydrogenases (ACADs) are a class of enzymes that function to catalyze the initial step in each cycle of fatty acid β-oxidation in the mitochondria of cells.

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Acyl-CoA

Acyl-CoA is a group of coenzymes involved in the metabolism of fatty acids.

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Amino acid

Amino acids are organic compounds containing amine (-NH2) and carboxyl (-COOH) functional groups, along with a side chain (R group) specific to each amino acid.

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Carnitine

Carnitine (β-hydroxy-γ-N-trimethylaminobutyric acid, 3-hydroxy-4-N,N,N- trimethylaminobutyrate) is a quaternary ammonium compound involved in metabolism in most mammals, plants and some bacteria. Carnitine may exist in two isomers, labeled D-carnitine and L-carnitine, as they are optically active. At room temperature, pure carnitine is a white powder, and a water-soluble zwitterion with low toxicity. Carnitine only exists in animals as the L-enantiomer, and D-carnitine is toxic because it inhibits the activity of L-carnitine. Carnitine, derived from an amino acid, is found in nearly all organisms and animal tissue. Carnitine is the generic expression for a number of compounds that include L-carnitine, acetyl-L-carnitine, and propionyl-L-carnitine. It is most accumulated in cardiac and skeletal muscles as it accounts for 0.1% of its dry matter. It was first derived from meat extracts in 1905, therefore the name carnitine is derived from Latin "carnus" or flesh. The body synthesizes enough carnitine from lysine side chains to keep up with the needs of energy production in the body as carnitine acts as a transporter of long-chain fatty acids into the mitochondria to be oxidized and produce energy. Some individuals with genetic or medical disorders (like preterm infants) cannot make enough, so this makes carnitine a conditionally essential nutrient for them.

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Coenzyme Q – cytochrome c reductase

The coenzyme Q: cytochrome c – oxidoreductase, sometimes called the cytochrome bc1 complex, and at other times complex III, is the third complex in the electron transport chain, playing a critical role in biochemical generation of ATP (oxidative phosphorylation).

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Coenzyme Q10

Coenzyme Q10, also known as ubiquinone, ubidecarenone, coenzyme Q, and abbreviated at times to CoQ10, CoQ, or Q10 is a coenzyme that is ubiquitous in animals and most bacteria (hence the name ubiquinone).

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Cysteine

Cysteine (symbol Cys or C) is a semi-essential proteinogenic amino acid with the formula HO2CCH(NH2)CH2SH.

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Disproportionation

Disproportionation, sometimes called dismutation, is a redox reaction in which a compound of intermediate oxidation state converts to two different compounds, one of higher and one of lower oxidation states.

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Electron transport chain

An electron transport chain (ETC) is a series of complexes that transfer electrons from electron donors to electron acceptors via redox (both reduction and oxidation occurring simultaneously) reactions, and couples this electron transfer with the transfer of protons (H+ ions) across a membrane.

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Electron-transferring flavoprotein

An electron transfer flavoprotein (ETF) or electron transfer flavoprotein complex (CETF) is a flavoprotein located on the matrix face of the inner mitochondrial membrane and functions as a specific electron acceptor for primary dehydrogenases, transferring the electrons to terminal respiratory systems such as electron-transferring-flavoprotein dehydrogenase.

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ETFDH

Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial is an enzyme that in humans is encoded by the ETFDH gene.

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Fatty acid

In chemistry, particularly in biochemistry, a fatty acid is a carboxylic acid with a long aliphatic chain, which is either saturated or unsaturated.

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Flavin adenine dinucleotide

In biochemistry, flavin adenine dinucleotide (FAD) is a redox cofactor, more specifically a prosthetic group of a protein, involved in several important enzymatic reactions in metabolism.

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Flavin group

Flavin (from Latin flavus, "yellow") is the common name for a group of organic compounds based on pteridine, formed by the tricyclic heterocycle isoalloxazine.

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Genetic disorder

A genetic disorder is a genetic problem caused by one or more abnormalities in the genome.

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Glutaric acidemia type 2

Glutaric acidemia type 2 is an autosomal recessive metabolic disorder that is characterised by defects in the ability of the body to use proteins and fats for energy.

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Hypoglycemia

Hypoglycemia, also known as low blood sugar, is when blood sugar decreases to below normal levels.

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Inner mitochondrial membrane

The inner mitochondrial membrane (IMM) is the mitochondrial membrane which separates the mitochondrial matrix from the intermembrane space.

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Iron–sulfur protein

Iron–sulfur proteins are proteins characterized by the presence of iron–sulfur clusters containing sulfide-linked di-, tri-, and tetrairon centers in variable oxidation states.

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Metabolism

Metabolism (from μεταβολή metabolē, "change") is the set of life-sustaining chemical transformations within the cells of organisms.

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Microbial metabolism

Microbial metabolism is the means by which a microbe obtains the energy and nutrients (e.g. carbon) it needs to live and reproduce.

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Mitochondrial matrix

In the mitochondrion, the matrix is the space within the inner membrane.

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Octanoyl-CoA

Octanoyl-coenzyme A is the endpoint of beta oxidation in peroxisomes.

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Oxidative phosphorylation

Oxidative phosphorylation (or OXPHOS in short) (UK, US) is the metabolic pathway in which cells use enzymes to oxidize nutrients, thereby releasing energy which is used to produce adenosine triphosphate (ATP).

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Riboflavin

Riboflavin, also known as vitamin B2, is a vitamin found in food and used as a dietary supplement.

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Semiquinone

Semiquinone (or ubisemiquinone) is a free radical resulting from the removal of one hydrogen atom with its electron during the process of dehydrogenation of a hydroquinone, such as hydroquinone itself or catechol, to a quinone or alternatively the addition of a single H atom to a quinone.

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Spectrophotometry

In chemistry, spectrophotometry is the quantitative measurement of the reflection or transmission properties of a material as a function of wavelength.

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Ubiquinol

Ubiquinol is an electron-rich (reduced) form of coenzyme Q10.

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Redirects here:

EC 1.5.5.1, ETF dehydrogenase, ETFD, Electron transfer flavoprotein-Q oxidoreductase, Electron transferflavoprotein-Q oxidoreductase, Electron transferring flavoprotein dehydrogenase, Electron-transferring flavoprotein dehydrogenase, Electron-transferring-flavoprotein:ubiquinone oxidoreductase, Flavoprotein : coenzyme Q oxidoreductase.

References

[1] https://en.wikipedia.org/wiki/Electron-transferring-flavoprotein_dehydrogenase

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