Electron-transferring-flavoprotein dehydrogenase

Information about Electron-transferring-flavoprotein dehydrogenase

Ribbon diagram of electron-transferring-flavoprotein dehydrogenase in complex with ubiquinone (bottom).
Identifiers
SymbolETFD
Alt. SymbolsETF-QO
Entrez&rn=1 2110
HUGO3483
OMIM&rn=1 231675
PDB2GMH
RefSeq&rn=1 NM_004453
UniProtQ16134
Other data
EC number1.5.5.1
LocusChr. 4 q 4q32.1
Electron-transferring-flavoprotein dehydrogenase (ETF dehydrogenase or electron transfer flavoprotein-ubiquinone oxidoreductase, EC 1.5.5.1) is an enzyme that transfers electrons from electron-transferring flavoprotein in the mitochondrial matrix, to the ubiquinone pool in the inner mitochondrial membrane.[1] It is part of the electron transport chain. The enzyme is found in both prokaryotes and eukaryotes and contains a flavin and FE-S cluster.[2] Deficiency in EFT dehydrogenase causes the human genetic disease multiple acyl-CoA dehydrogenase deficiency.[3]

Reduced electron-transferring flavoprotein + ubiquinone <=> electron-transferring flavoprotein + ubiquinol

See also

References

1. ^ Ramsay RR, Steenkamp DJ, Husain M (1987). "Reactions of electron-transfer flavoprotein and electron-transfer flavoprotein: ubiquinone oxidoreductase". Biochem. J. 241 (3): 883-92. PMID 3593226. 
2. ^ Zhang J, Frerman FE, Kim JJ (2006). "Structure of electron transfer flavoprotein-ubiquinone oxidoreductase and electron transfer to the mitochondrial ubiquinone pool". Proc. Natl. Acad. Sci. U.S.A. 103 (44): 16212-7. DOI:10.1073/pnas.0604567103. PMID 17050691. 
3. ^ Vianey-Liaud C, Divry P, Gregersen N, Mathieu M (1987). "The inborn errors of mitochondrial fatty acid oxidation". J. Inherit. Metab. Dis. 10 Suppl 1: 159-200. PMID 3119938. 

External links

 •  • [ e]
CH-NH oxidoreductases (EC 1.5)
1.5.1 - NAD or NADP acceptorDihydrofolate reductase - Saccharopine dehydrogenase - Methylenetetrahydrofolate reductase
1.5.3 - oxygen acceptorDihydrobenzophenanthridine oxidase - Sarcosine oxidase
1.5.5 - quinone acceptorElectron-transferring-flavoprotein dehydrogenase
 •  • [ e]
Mitochondrial electron transport chain/oxidative phosphorylation
Complex I - Complex II - Coenzyme Q - Complex III - Cytochrome C - Complex IV - Alternative oxidase - Electron-transferring-flavoprotein dehydrogenase
 •  • [ e]
Cellular Respiration
Aerobic RespirationGlycolysis → Pyruvate Decarboxylation → Citric Acid Cycle → Oxidative Phosphorylation (Electron Transport Chain + ATP synthase)
Anaerobic RespirationGlycolysis → Lactic Acid Formation or Ethanol Formation
Coenzyme Q10 (also known as ubiquinone, ubidecarenone, coenzyme Q, and abbreviated at times to CoQ10, CoQ, Q10, or Q) is a benzoquinone, where Q refers to the quinone chemical group, and 10 refers to the isoprenyl chemical subunits.
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The Entrez Global Query Cross-Database Search System is a powerful federated search engine, or web portal that allows users to search many discrete health sciences databases at the National Center for Biotechnology Information (NCBI) website.
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Hugo is a male given name, a latinized form of the name Hugh, a German/Teutonic name meaning "Bright in Mind and Spirit".

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Protein Data Bank (PDB) is a repository for 3-D structural data of proteins and nucleic acids. This data, typically obtained by X-ray crystallography or NMR spectroscopy, is submitted by biologists and biochemists from around the world, is released into the public domain,
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Enzyme Commission number (EC number) is a numerical classification scheme for enzymes, based on the chemical reactions they catalyze. As a system of enzyme nomenclature, every EC number is associated with a recommended name for the respective enzyme.
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locus (plural loci) is a fixed position on a chromosome, such as the position of a gene or a biomarker (genetic marker). A variant of the DNA sequence at a given locus is called an allele. The ordered list of loci known for a particular genome is called a genetic map.
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Electron transfer flavoproteins (ETFs) serve as specific electron acceptors for primary dehydrogenases, transferring the electrons to terminal respiratory systems such as electron-transferring-flavoprotein dehydrogenase.
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matrix contains soluble enzymes that catalyze the oxidation of pyruvate and other small organic molecules.

The mitochondrial matrix also contains the mitochondria's DNA and ribosomes.
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Coenzyme Q10 (also known as ubiquinone, ubidecarenone, coenzyme Q, and abbreviated at times to CoQ10, CoQ, Q10, or Q) is a benzoquinone, where Q refers to the quinone chemical group, and 10 refers to the isoprenyl chemical subunits.
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mitochondrial inner membrane forms internal compartments known as cristae, which allow greater space for the proteins such as cytochromes to function properly and efficiently. The electron transport chain is located on the inner membrane of the mitochondria.
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electron transport chain associates electron carriers (such as NAD+ and FADH2) and mediating biochemical reactions that produce adenosine triphosphate (ATP), which is the energy currency of life.
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Flavin is a tricyclic heteronuclear organic ring based on pteridine whose biochemical source is the vitamin riboflavin. The flavin moiety is often attached with an adenosine diphosphate to form flavin adenine dinucleotide (FAD
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Iron-sulfur proteins are proteins characterized by the presence of polymetallic systems (iron-sulfur clusters) containing sulfide ions, in which the iron ions have variable oxidation states.
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A genetic disorder is a condition caused by abnormalities in genes or chromosomes. While some diseases, such as cancer, are due to genetic abnormalities acquired in a few cells during life, the term "genetic disease" most commonly refers to diseases present in all cells of the body
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Oxidative phosphorylation is a metabolic pathway that uses energy released by the oxidation of nutrients to produce adenosine triphosphate (ATP). Although the many forms of life on Earth utilize a range of different nutrients, almost all carry out oxidative phosphorylation to
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electron transport chain associates electron carriers (such as NAD+ and FADH2) and mediating biochemical reactions that produce adenosine triphosphate (ATP), which is the energy currency of life.
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Microbial metabolism is the means by which a microbe obtains the energy and nutrients (e.g. carbon) it needs to live and reproduce. Microbes use many different types of metabolic strategies and species can often be differentiated from each other based on metabolic characteristics.
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Metabolism is the complete set of chemical reactions that occur in living cells. These processes are the basis of life, allowing cells to grow and reproduce, maintain their structures, and respond to their environments. Metabolism is usually divided into two categories.
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digital object identifier (or DOI) is a permanent identifier given to a document, which is not related to its current location. A typical use of a DOI is to give a scientific paper or article a unique identifying number that can be used by anyone to locate details of the paper, and
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In biochemistry, an oxidoreductase is an enzyme that catalyzes the transfer of electrons from one molecule (the reductant, also called the hydrogen acceptor or electron donor) to another (the oxidant, also called the hydrogen donor or electron acceptor).
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Enzyme Commission number (EC number) is a numerical classification scheme for enzymes, based on the chemical reactions they catalyze. As a system of enzyme nomenclature, every EC number is associated with a recommended name for the respective enzyme.
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Nicotinamide adenine dinucleotide (NAD+ or in older notation DPN+) is an important coenzyme found in cells. It plays key roles as a carrier of electrons and a participant in metabolic redox reactions, as well as in cell signaling.
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Nicotinamide adenine dinucleotide phosphate (NADP) is used in anabolic reactions, such as fatty acid and nucleic acid synthesis, which require NADPH as a reducing agent.

NADPH is the reduced form of NADP+, and NADP+ is the oxidized form of NADPH.
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Dihydrofolate reductase, or DHFR, reduces dihydrofolic acid to tetrahydrofolic acid, using NADPH as electron donor, which can be converted to the kinds of tetrahydrofolate cofactors used in 1-carbon transfer chemistry.
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Saccharopine dehydrogenase is an enzyme involved in the metabolism of lysine, via saccharopine.

It can be classified under EC 1.5.1.7 , EC 1.5.1.8 , EC 1.5.1.9 , and EC 1.5.1.10 .

Pathology

Deficiencies are associated with hyperlysinemia.
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Methylenetetrahydrofolate reductase (MTHFR) is an enzyme (EC 1.5.1.20 ) that exists in the cytoplasm of cells. MTHFR contains a bound flavin cofactor and uses NAD(P)H as the reducing agent.
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2, −1
(neutral oxide)
Electronegativity 3.44 (Pauling scale)
Ionization energies
(more) 1st: 1313.9 kJmol−1
2nd: 3388.3 kJmol−1
3rd: 5300.5 kJmol−1

Atomic radius 60 pm
Atomic radius (calc.
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Dihydrobenzophenanthridine oxidase (DHBP oxidase) is an enzyme. In the IUBMB Enzyme Nomenclature, Dihydrobenzophenanthridine oxidase is EC 1.5.3.12 . Dihydrobenzophenanthridine oxidase/EC 1.5.3.
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