Acyl coa dehydrogenase long chain. Medical search

Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in the presence of NAD. They are key enzymes in the oxidation of fatty acids and in mitochondrial fatty acid synthesis.

Enzymes that catalyze the first step in the beta-oxidation of FATTY ACIDS.

A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.

S-Acyl coenzyme A. Fatty acid coenzyme A derivatives that are involved in the biosynthesis and oxidation of fatty acids as well as in ceramide formation.

An enzyme that catalyses the last step of the TRIACYLGLYCEROL synthesis reaction in which diacylglycerol is covalently joined to LONG-CHAIN ACYL COA to form triglyceride. It was formerly categorized as EC 2.3.1.124.

An enzyme that catalyzes the formation of cholesterol esters by the direct transfer of the fatty acid group from a fatty acyl CoA derivative. This enzyme has been found in the adrenal gland, gonads, liver, intestinal mucosa, and aorta of many mammalian species. EC 2.3.1.26.

A tetrameric enzyme that, along with the coenzyme NAD+, catalyzes the interconversion of LACTATE and PYRUVATE. In vertebrates, genes for three different subunits (LDH-A, LDH-B and LDH-C) exist.

Enzymes from the transferase class that catalyze the transfer of acyl groups from donor to acceptor, forming either esters or amides. (From Enzyme Nomenclature 1992) EC 2.3.

A zinc-containing enzyme which oxidizes primary and secondary alcohols or hemiacetals in the presence of NAD. In alcoholic fermentation, it catalyzes the final step of reducing an aldehyde to an alcohol in the presence of NADH and hydrogen.

Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)

Enzymes that catalyze the dehydrogenation of GLYCERALDEHYDE 3-PHOSPHATE. Several types of glyceraldehyde-3-phosphate-dehydrogenase exist including phosphorylating and non-phosphorylating varieties and ones that transfer hydrogen to NADP and ones that transfer hydrogen to NAD.

Enzymes that catalyze the formation of acyl-CoA derivatives. EC 6.2.1.

An enzyme that oxidizes an aldehyde in the presence of NAD+ and water to an acid and NADH. This enzyme was formerly classified as EC 1.1.1.70.

An enzyme that catalyzes the conversion of L-glutamate and water to 2-oxoglutarate and NH3 in the presence of NAD+. (From Enzyme Nomenclature, 1992) EC 1.4.1.2.

An enzyme that catalyzes the conversion of (S)-malate and NAD+ to oxaloacetate and NADH. EC 1.1.1.37.

An enzyme of the oxidoreductase class that catalyzes the conversion of isocitrate and NAD+ to yield 2-ketoglutarate, carbon dioxide, and NADH. It occurs in cell mitochondria. The enzyme requires Mg2+, Mn2+; it is activated by ADP, citrate, and Ca2+, and inhibited by NADH, NADPH, and ATP. The reaction is the key rate-limiting step of the citric acid (tricarboxylic) cycle. (From Dorland, 27th ed) (The NADP+ enzyme is EC 1.1.1.42.) EC 1.1.1.41.

A subclass of enzymes which includes all dehydrogenases acting on primary and secondary alcohols as well as hemiacetals. They are further classified according to the acceptor which can be NAD+ or NADP+ (subclass 1.1.1), cytochrome (1.1.2), oxygen (1.1.3), quinone (1.1.5), or another acceptor (1.1.99).

Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.

An enzyme that catalyzes the first and rate-determining steps of peroxisomal beta-oxidation of fatty acids. It acts on COENZYME A derivatives of fatty acids with chain lengths from 8 to 18, using FLAVIN-ADENINE DINUCLEOTIDE as a cofactor.

Compounds with three contiguous nitrogen atoms in linear format, H2N-N=NH, and hydrocarbyl derivatives.

The rate dynamics in chemical or physical systems.

An 86-amino acid polypeptide, found in central and peripheral tissues, that displaces diazepam from the benzodiazepine recognition site on the gamma-aminobutyric acid receptor (RECEPTORS, GABA). It also binds medium- and long-chain acyl-CoA esters and serves as an acyl-CoA transporter. This peptide regulates lipid metabolism.

A fatty acid coenzyme derivative which plays a key role in fatty acid oxidation and biosynthesis.

A flavoprotein containing oxidoreductase that catalyzes the reduction of lipoamide by NADH to yield dihydrolipoamide and NAD+. The enzyme is a component of several MULTIENZYME COMPLEXES.

A flavoprotein containing oxidoreductase that catalyzes the dehydrogenation of SUCCINATE to fumarate. In most eukaryotic organisms this enzyme is a component of mitochondrial electron transport complex II.

The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.

Reversibly catalyze the oxidation of a hydroxyl group of carbohydrates to form a keto sugar, aldehyde or lactone. Any acceptor except molecular oxygen is permitted. Includes EC 1.1.1.; EC 1.1.2.; and 1.1.99.

An alcohol oxidoreductase which catalyzes the oxidation of L-iditol to L-sorbose in the presence of NAD. It also acts on D-glucitol to form D-fructose. It also acts on other closely related sugar alcohols to form the corresponding sugar. EC 1.1.1.14

The class of all enzymes catalyzing oxidoreduction reactions. The substrate that is oxidized is regarded as a hydrogen donor. The systematic name is based on donor:acceptor oxidoreductase. The recommended name will be dehydrogenase, wherever this is possible; as an alternative, reductase can be used. Oxidase is only used in cases where O2 is the acceptor. (Enzyme Nomenclature, 1992, p9)

A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.

Oxidoreductases that are specific for ALDEHYDES.

A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)

A flavoprotein oxidoreductase that has specificity for long-chain fatty acids. It forms a complex with ELECTRON-TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.

A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).

A glucose dehydrogenase that catalyzes the oxidation of beta-D-glucose to form D-glucono-1,5-lactone, using NAD as well as NADP as a coenzyme.

Enzymes of the oxidoreductase class that catalyze the dehydrogenation of hydroxysteroids. (From Enzyme Nomenclature, 1992) EC 1.1.-.

FATTY ACIDS in which the carbon chain contains one or more double or triple carbon-carbon bonds.

A group of 16-carbon fatty acids that contain no double bonds.

A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.

Reversibly catalyzes the oxidation of a hydroxyl group of sugar alcohols to form a keto sugar, aldehyde or lactone. Any acceptor except molecular oxygen is permitted. Includes EC 1.1.1.; EC 1.1.2. and EC 1.1.99.

Catalyze the oxidation of 3-hydroxysteroids to 3-ketosteroids.

D-Glucose:1-oxidoreductases. Catalyzes the oxidation of D-glucose to D-glucono-gamma-lactone and reduced acceptor. Any acceptor except molecular oxygen is permitted. Includes EC 1.1.1.47; EC 1.1.1.118; EC 1.1.1.119 and EC 1.1.99.10.

Fatty acid esters of cholesterol which constitute about two-thirds of the cholesterol in the plasma. The accumulation of cholesterol esters in the arterial intima is a characteristic feature of atherosclerosis.

An enzyme of the oxidoreductase class that catalyzes the reaction 6-phospho-D-gluconate and NADP+ to yield D-ribulose 5-phosphate, carbon dioxide, and NADPH. The reaction is a step in the pentose phosphate pathway of glucose metabolism. (From Dorland, 27th ed) EC 1.1.1.43.

A flavoprotein and iron sulfur-containing oxidoreductase that catalyzes the oxidation of NADH to NAD. In eukaryotes the enzyme can be found as a component of mitochondrial electron transport complex I. Under experimental conditions the enzyme can use CYTOCHROME C GROUP as the reducing cofactor. The enzyme was formerly listed as EC 1.6.2.1.

A group of fatty acids that contain 18 carbon atoms and a double bond at the omega 9 carbon.

The process of converting an acid into an alkyl or aryl derivative. Most frequently the process consists of the reaction of an acid with an alcohol in the presence of a trace of mineral acid as catalyst or the reaction of an acyl chloride with an alcohol. Esterification can also be accomplished by enzymatic processes.

Artifactual vesicles formed from the endoplasmic reticulum when cells are disrupted. They are isolated by differential centrifugation and are composed of three structural features: rough vesicles, smooth vesicles, and ribosomes. Numerous enzyme activities are associated with the microsomal fraction. (Glick, Glossary of Biochemistry and Molecular Biology, 1990; from Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)

An enzyme that catalyzes the dehydrogenation of inosine 5'-phosphate to xanthosine 5'-phosphate in the presence of NAD. EC 1.1.1.205.

The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.

Alcohol oxidoreductases with substrate specificity for LACTIC ACID.

In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.

Flavoproteins that catalyze reversibly the reduction of carbon dioxide to formate. Many compounds can act as acceptors, but the only physiologically active acceptor is NAD. The enzymes are active in the fermentation of sugars and other compounds to carbon dioxide and are the key enzymes in obtaining energy when bacteria are grown on formate as the main carbon source. They have been purified from bovine blood. EC 1.2.1.2.

Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.

A class of enzymes that catalyzes the oxidation of 17-hydroxysteroids to 17-ketosteroids. EC 1.1.-.

Electron-dense cytoplasmic particles bounded by a single membrane, such as PEROXISOMES; GLYOXYSOMES; and glycosomes.

An enzyme that catalyzes the oxidation of XANTHINE in the presence of NAD+ to form URIC ACID and NADH. It acts also on a variety of other purines and aldehydes.

A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.

The addition of an organic acid radical into a molecule.

The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.

Oxidoreductases that are specific for KETONES.

An enzyme that catalyzes reversibly the conversion of palmitoyl-CoA to palmitoylcarnitine in the inner mitochondrial membrane. EC 2.3.1.21.

The E1 component of the multienzyme PYRUVATE DEHYDROGENASE COMPLEX. It is composed of 2 alpha subunits (pyruvate dehydrogenase E1 alpha subunit) and 2 beta subunits (pyruvate dehydrogenase E1 beta subunit).

Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)

A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.

The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.

RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.

Hydroxysteroid dehydrogenases that catalyzes the reversible conversion of CORTISOL to the inactive metabolite CORTISONE. Enzymes in this class can utilize either NAD or NADP as cofactors.

Acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.

A generic term for fats and lipoids, the alcohol-ether-soluble constituents of protoplasm, which are insoluble in water. They comprise the fats, fatty oils, essential oils, waxes, phospholipids, glycolipids, sulfolipids, aminolipids, chromolipids (lipochromes), and fatty acids. (Grant & Hackh's Chemical Dictionary, 5th ed)

An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).

An enzyme that catalyzes the oxidation of UDPglucose to UDPglucuronate in the presence of NAD+. EC 1.1.1.22.

An oxidoreductase involved in pyrimidine base degradation. It catalyzes the catabolism of THYMINE; URACIL and the chemotherapeutic drug, 5-FLUOROURACIL.

A flavoprotein oxidoreductase that has specificity for short-chain fatty acids. It forms a complex with ELECTRON-TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.

A disease-producing enzyme deficiency subject to many variants, some of which cause a deficiency of GLUCOSE-6-PHOSPHATE DEHYDROGENASE activity in erythrocytes, leading to hemolytic anemia.

The parts of a macromolecule that directly participate in its specific combination with another molecule.

A low-affinity 11 beta-hydroxysteroid dehydrogenase found in a variety of tissues, most notably in LIVER; LUNG; ADIPOSE TISSUE; vascular tissue; OVARY; and the CENTRAL NERVOUS SYSTEM. The enzyme acts reversibly and can use either NAD or NADP as cofactors.

Structurally related forms of an enzyme. Each isoenzyme has the same mechanism and classification, but differs in its chemical, physical, or immunological characteristics.

The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.

An NAD-dependent enzyme that catalyzes the reversible DEAMINATION of L-ALANINE to PYRUVATE and AMMONIA. The enzyme is needed for growth when ALANINE is the sole CARBON or NITROGEN source. It may also play a role in CELL WALL synthesis because L-ALANINE is an important constituent of the PEPTIDOGLYCAN layer.

A 3-hydroxysteroid dehydrogenase which catalyzes the reversible reduction of the active androgen, DIHYDROTESTOSTERONE to 5 ALPHA-ANDROSTANE-3 ALPHA,17 BETA-DIOL. It also has activity towards other 3-alpha-hydroxysteroids and on 9-, 11- and 15- hydroxyprostaglandins. The enzyme is B-specific in reference to the orientation of reduced NAD or NADPH.

Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.

Sugar alcohol dehydrogenases that have specificity for MANNITOL. Enzymes in this category are generally classified according to their preference for a specific reducing cofactor.

A constituent of STRIATED MUSCLE and LIVER. It is an amino acid derivative and an essential cofactor for fatty acid metabolism.

Chromatography on thin layers of adsorbents rather than in columns. The adsorbent can be alumina, silica gel, silicates, charcoals, or cellulose. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)

Catalyzes reversibly the oxidation of hydroxyl groups of prostaglandins.

The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)

A class of membrane lipids that have a polar head and two nonpolar tails. They are composed of one molecule of the long-chain amino alcohol sphingosine (4-sphingenine) or one of its derivatives, one molecule of a long-chain acid, a polar head alcohol and sometimes phosphoric acid in diester linkage at the polar head group. (Lehninger et al, Principles of Biochemistry, 2nd ed)

Systems of enzymes which function sequentially by catalyzing consecutive reactions linked by common metabolic intermediates. They may involve simply a transfer of water molecules or hydrogen atoms and may be associated with large supramolecular structures such as MITOCHONDRIA or RIBOSOMES.

A metalloflavoprotein enzyme involved the metabolism of VITAMIN A, this enzyme catalyzes the oxidation of RETINAL to RETINOIC ACID, using both NAD+ and FAD coenzymes. It also acts on both the 11-trans- and 13-cis-forms of RETINAL.

Fats present in food, especially in animal products such as meat, meat products, butter, ghee. They are present in lower amounts in nuts, seeds, and avocados.

The sum of the weight of all the atoms in a molecule.

Closed vesicles of fragmented endoplasmic reticulum created when liver cells or tissue are disrupted by homogenization. They may be smooth or rough.

A group of enzymes that catalyze the reversible reduction-oxidation reaction of 20-hydroxysteroids, such as from a 20-ketosteroid to a 20-alpha-hydroxysteroid (EC 1.1.1.149) or to a 20-beta-hydroxysteroid (EC 1.1.1.53).

Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.

An high-affinity, NAD-dependent 11-beta-hydroxysteroid dehydrogenase that acts unidirectionally to catalyze the dehydrogenation of CORTISOL to CORTISONE. It is found predominantly in mineralocorticoid target tissues such as the KIDNEY; COLON; SWEAT GLANDS; and the PLACENTA. Absence of the enzyme leads to a fatal form of childhood hypertension termed, APPARENT MINERALOCORTICOID EXCESS SYNDROME.

A mitochondrial flavoprotein, this enzyme catalyzes the oxidation of 3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA using FAD as a cofactor. Defects in the enzyme, is associated with isovaleric acidemia (IVA).

Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)

Proteins prepared by recombinant DNA technology.

A broad category of membrane transport proteins that specifically transport FREE FATTY ACIDS across cellular membranes. They play an important role in LIPID METABOLISM in CELLS that utilize free fatty acids as an energy source.

Fractionation of a vaporized sample as a consequence of partition between a mobile gaseous phase and a stationary phase held in a column. Two types are gas-solid chromatography, where the fixed phase is a solid, and gas-liquid, in which the stationary phase is a nonvolatile liquid supported on an inert solid matrix.

An enzyme that catalyzes the reduction of aspartic beta-semialdehyde to homoserine, which is the branch point in biosynthesis of methionine, lysine, threonine and leucine from aspartic acid. EC 1.1.1.3.

Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death. (1/138)

BACKGROUND: Genetic defects are being increasingly recognized in the etiology of primary cardiomyopathy (CM). Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the first step in the beta-oxidation spiral of fatty acid metabolism, the crucial pathway for cardiac energy production. METHODS AND RESULTS: We studied 37 patients with CM, nonketotic hypoglycemia and hepatic dysfunction, skeletal myopathy, or sudden death in infancy with hepatic steatosis, features suggestive of fatty acid oxidation disorders. Single-stranded conformational variance was used to screen genomic DNA. DNA sequencing and mutational analysis revealed 21 different mutations on the VLCAD gene in 18 patients. Of the mutations, 80% were associated with CM. Severe CM in infancy was recognized in most patients (67%) at presentation. Hepatic dysfunction was common (33%). RNA blot analysis and VLCAD enzyme assays showed a severe reduction in VLCAD mRNA in patients with frame-shift or splice-site mutations and absent or severe reduction in enzyme activity in all. CONCLUSIONS: Infantile CM is the most common clinical phenotype of VLCAD deficiency. Mutations in the human VLCAD gene are heterogeneous. Although mortality at presentation is high, both the metabolic disorder and cardiomyopathy are reversible. (+info)

The medium-/long-chain fatty acyl-CoA dehydrogenase (fadF) gene of Salmonella typhimurium is a phase 1 starvation-stress response (SSR) locus. (2/138)

Salmonella enterica serovar Typhimurium (S. typhimurium) is an enteric pathogen that causes significant morbidity in humans and other mammals. During their life cycle, salmonellae must survive frequent exposures to a variety of environmental stresses, e.g. carbon-source (C) starvation. The starvation-stress response (SSR) of S. typhimurium encompasses the genetic and physiological realignments that occur when an essential nutrient becomes limiting for bacterial growth. The function of the SSR is to produce a cell capable of surviving long-term starvation. This paper reports that three C-starvation-inducible lac fusions from an S. typhimurium C-starvation-inducible lac fusion library are all within a gene identified as fadF, which encodes an acyl-CoA dehydrogenase (ACDH) specific for medium-/long-chain fatty acids. This identification is supported by several findings: (a) significant homology at the amino acid sequence level with the ACDH enzymes from other bacteria and eukaryotes, (b) undetectable beta-oxidation levels in fadF insertion mutants, (c) inability of fad insertion mutants to grow on oleate or decanoate as a sole C-source, and (d) inducibility of fadF::lac fusions by the long-chain fatty acid oleate. In addition, the results indicate that the C-starvation-induction of fadF is under negative control by the FadR global regulator and positive control by the cAMP:cAMP receptor protein complex and ppGpp. It is also shown that the fadF locus is important for C-starvation-survival in S. typhimurium. Furthermore, the results demonstrate that fadF is induced within cultured Madin-Darby canine kidney (MDCK) epithelial cells, suggesting that signals for its induction (C-starvation and/or long-chain fatty acids) may be present in the intracellular environment encountered by S. typhimurium. However, fadF insertion mutations did not have an overt effect on mouse virulence. (+info)

Cloning and mapping of three pig acyl-CoA dehydrogenase genes. (3/138)

To investigate the structure of porcine genes involved in the beta-oxidation of fatty acid, we isolated the short-chain acyl-CoA dehydrogenase (SCAD), medium-chain acyl-CoA dehydrogenase (MCAD), and long-chain acyl-CoA dehydrogenase (LCAD) genes from the pig. The cDNA of SCAD, MCAD and LCAD genes were 1899 bp, 1835 bp 1835 bp and 1704 bp long and coded for 413-aa, 422-aa and 430-aa precursor proteins, respectively. Three genes, SCAD, MCAD and LCAD were mapped to 14p16.2-23.2, 6q32.4-33, and 15q24.2-26.3, respectively. (+info)

Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. (4/138)

BACKGROUND: The clinical manifestations of inherited disorders of fatty acid oxidation vary according to the enzymatic defect. They may present as isolated cardiomyopathy, sudden death, progressive skeletal myopathy, or hepatic failure. Arrhythmia is an unusual presenting symptom of fatty acid oxidation deficiencies. METHODS AND RESULTS: Over a period of 25 years, 107 patients were diagnosed with an inherited fatty acid oxidation disorder. Arrhythmia was the predominant presenting symptom in 24 cases. These 24 cases included 15 ventricular tachycardias, 4 atrial tachycardias, 4 sinus node dysfunctions with episodes of atrial tachycardia, 6 atrioventricular blocks, and 4 left bundle-branch blocks in newborn infants. Conduction disorders and atrial tachycardias were observed in patients with defects of long-chain fatty acid transport across the inner mitochondrial membrane (carnitine palmitoyl transferase type II deficiency and carnitine acylcarnitine translocase deficiency) and in patients with trifunctional protein deficiency. Ventricular tachycardias were observed in patients with any type of fatty acid oxidation deficiency. Arrhythmias were absent in patients with primary carnitine carrier, carnitine palmitoyl transferase I, and medium chain acyl coenzyme A dehydrogenase deficiencies. CONCLUSIONS: The accumulation of arrhythmogenic intermediary metabolites of fatty acids, such as long-chain acylcarnitines, may be responsible for arrhythmias. Inborn errors of fatty acid oxidation should be considered in unexplained sudden death or near-miss in infants and in infants with conduction defects or ventricular tachycardia. Diagnosis can be easily ascertained by an acylcarnitine profile from blood spots on filter paper. (+info)

Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids. (5/138)

The first reaction of mitochondrial beta-oxidation, which is catalyzed by acyl-CoA dehydrogenases, was studied with unsaturated fatty acids that have a double bond either at the 4,5 or 5,6 position. The CoA thioesters of docosahexaenoic acid, arachidonic acid, 4,7,10-cis-hexadecatrienoic acid, 5-cis-tetradecenoic acid, and 4-cis-decenoic acid were effectively dehydrogenated by both rat and human long-chain acyl-CoA dehydrogenases (LCAD), whereas they were poor substrates of very long-chain acyl-CoA dehydrogenases (VLCAD). VLCAD, however, was active with CoA derivatives of long-chain saturated fatty acids or unsaturated fatty acids that have double bonds further removed from the thioester function. Although bovine LCAD effectively dehydrogenated 5-cis-tetradecenoyl-CoA (14:1) and 4,7,10-cis-hexadecatrienoyl-CoA, it was nearly inactive toward the other unsaturated substrates. The catalytic efficiency of rat VLCAD with 14:1 as substrate was only 4% of the efficiency determined with tetradecanoyl-CoA, whereas LCAD acted equally well on both substrates. The conclusion of this study is that LCAD serves an important, if not essential function in the beta-oxidation of unsaturated fatty acids. (+info)

Effect of endurance training on lipid metabolism in women: a potential role for PPARalpha in the metabolic response to training. (6/138)

Endurance training increases fatty acid oxidation (FAO) and skeletal muscle oxidative capacity. However, the source of the additional fat and the mechanisms for increasing FAO capacity in muscle are not clear. We measured whole body and regional lipolytic activity and whole body and plasma FAO in six lean women during 90 min of bicycling exercise (50% pretraining peak O(2) consumption) before and after 12 wk of endurance training. We also assessed skeletal muscle content of peroxisome proliferator-activated receptor-alpha (PPARalpha) and its target proteins that regulate FAO [medium-chain and very long chain acyl-CoA dehydrogenase (MCAD and VLCAD)]. Despite a 25% increase in whole body FAO during exercise after training (P < 0.05), training did not alter regional adipose tissue lipolysis (abdominal: 0.56 +/- 0.26 and 0.57 +/- 0.10 micromol x 100 g(-1) x min(-1); femoral: 0.13 +/- 0.07 and 0.09 +/- 0.02 micromol x 100 g(-1) x min(-1)), whole body palmitate rate of appearance in plasma (168 +/- 18 and 150 +/- 25 micromol/min), and plasma FAO (554 +/- 61 and 601 +/- 45 micromol/min). However, training doubled the levels of muscle PPARalpha, MCAD, and VLCAD. We conclude that training increases the use of nonplasma fatty acids and may enhance skeletal muscle oxidative capacity by PPARalpha regulation of gene expression. (+info)

Mitochondrial transcription factor A is increased but expression of ATP synthase beta subunit and medium-chain acyl-CoA dehydrogenase genes are decreased in hearts of copper-deficient rats. (7/138)

The mechanism(s) by which impaired mitochondrial respiratory function and the accumulation of lipid droplets and mitochondria in hearts of copper-deficient rats occur remains unclear. It is not known whether specific components of the regulatory pathway involved in mitochondrial biogenesis, such as mitochondrial transcription factor A (mtTFA) and nuclear respiratory factors 1 and 2 (NRF-1 and NRF-2), are activated in copper deficiency. Little is known about gene expression of enzymes involved in fatty acid oxidation (FAO) in hearts of copper-deficient rats. Male weanling rats were fed copper-adequate (CuA), copper-deficient (CuD) or pair-fed (CuP) diets for 5 wk. Mitochondria and lipid droplet volume densities from electron micrographs were greater and there was an elevation in the mtTFA protein level in hearts of copper-deficient rats. DNA binding activities of NRF-1 and NRF-2 did not differ among the groups. Northern blot analysis of cardiac tissue revealed that transcripts of F(1)F(0)-ATP synthase subunit c were greater, but mRNA levels of ATP synthase beta subunit and the FAO enzyme, medium-chain acyl-CoA dehydrogenase (MCAD), were lower in hearts of copper-deficient rats. Long-chain acyl-CoA dehydrogenase (LCAD) mRNA levels did not differ among treatment groups. These results suggest that certain components of the mitochondrial biogenesis program are activated in hearts of copper-deficient rats. F(1)F(0)-ATP synthase beta subunit and MCAD transcript levels remain low, which may contribute to impaired mitochondrial respiratory function, decreased fatty acid utilization and lipid droplet accumulation in hearts of copper-deficient rats. (+info)

Production of fatty acid components of meadowfoam oil in somatic soybean embryos. (8/138)

The seed oil of meadowfoam (Limnanthes alba) and other Limnanthes spp. is enriched in the unusual fatty acid Delta(5)-eicosenoic acid (20:1Delta(5)). This fatty acid has physical and chemical properties that make the seed oil of these plants useful for a number of industrial applications. An expressed sequence tag approach was used to identify cDNAs for enzymes involved in the biosynthesis of 20:1Delta(5)). By random sequencing of a library prepared from developing Limnanthes douglasii seeds, a class of cDNAs was identified that encode a homolog of acyl-coenzyme A (CoA) desaturases found in animals, fungi, and cyanobacteria. Expression of a cDNA for the L. douglasii acyl-CoA desaturase homolog in somatic soybean (Glycine max) embryos behind a strong seed-specific promoter resulted in the accumulation of Delta(5)-hexadecenoic acid to amounts of 2% to 3% (w/w) of the total fatty acids of single embryos. Delta(5)-Octadecenoic acid and 20:1Delta(5) also composed <1% (w/w) each of the total fatty acids of these embryos. In addition, cDNAs were identified from the L. douglasii expressed sequence tags that encode a homolog of fatty acid elongase 1 (FAE1), a beta-ketoacyl-CoA synthase that catalyzes the initial step of very long-chain fatty acid synthesis. Expression of the L. douglassi FAE1 homolog in somatic soybean embryos was accompanied by the accumulation of C(20) and C(22) fatty acids, principally as eicosanoic acid, to amounts of 18% (w/w) of the total fatty acids of single embryos. To partially reconstruct the biosynthetic pathway of 20:1Delta(5) in transgenic plant tissues, cDNAs for the L. douglasii acyl-CoA desaturase and FAE1 were co-expressed in somatic soybean embryos. In the resulting transgenic embryos, 20:1Delta(5) and Delta(5)-docosenoic acid composed up to 12% of the total fatty acids. (+info)

... load and carnitine load on plasma long-chain acylcarnitine levels in mitochondrial very long-chain acyl-CoA dehydrogenase ... This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product ... "acyl-CoA dehydrogenase, very long chain". Strauss AW, Powell CK, Hale DE, Anderson MM, Ahuja A, Brackett JC, Sims HF (Nov 1995 ... Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) is an enzyme that in humans is encoded by the ACADVL ...

https://en.wikipedia.org/wiki/ACADVL

"Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), long-chain enoyl-CoA hydratase, and long-chain thiolase activities. ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ... CS1 maint: discouraged parameter (link) "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase ( ...

https://en.wikipedia.org/wiki/Mitochondrial_trifunctional_protein_deficiency

... acyl-CoA dehydrogenase, long chain - which is a member of the acyl-CoA dehydrogenase family. The acyl-CoA dehydrogenase family ... "Cardiac hypertrophy in mice with long-chain acyl-CoA dehydrogenase or very long-chain acyl-CoA dehydrogenase deficiency". ... Acyl-CoA dehydrogenase, long chain". Kurtz DM, Tolwani RJ, Wood PA (May 1998). "Structural characterization of the mouse long- ... Acyl-CoA dehydrogenase, long chain is a protein that in humans is encoded by the ACADL gene. ACADL is a gene that encodes LCAD ...

https://en.wikipedia.org/wiki/ACADL

... it interferes with the transport of long-chain fatty acids into the mitochondria. Also, it inhibits acyl-CoA dehydrogenases, so ...

https://en.wikipedia.org/wiki/Jamaican_vomiting_sickness

Acyl-CoA dehydrogenase, C-2 to C-3 short chain is an enzyme that in humans is encoded by the ACADS gene. This gene encodes a ... The coding sequence of this gene is 1239 bp long. The encoded protein has 412 amino acids, and its size is 44.3 kDa (Human) or ... "Entrez Gene: Acyl-CoA dehydrogenase, C-2 to C-3 short chain". Tein I, Elpeleg O, Ben-Zeev B, Korman SH, Lossos A, Lev D, Lerman ... As short-chain acyl-CoA dehydrogenase is involved in beta-oxidation, a deficiency in this enzyme is marked by an increased ...

https://en.wikipedia.org/wiki/ACADS

"Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain". Biochemical and ... typically C16-acylCoA and longer. It has been observed that ACAD9 can catalyze acyl-CoAs with very long chains. The specific ... "Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients". ... Acyl-CoA dehydrogenase family member 9, mitochondrial is an enzyme that in humans is encoded by the ACAD9 gene. Mitochondrial ...

https://en.wikipedia.org/wiki/ACAD9

"Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction". The Journal of Biological ... ACADM, acyl-CoA dehydrogenase, C-4 to C-12 straight chain, ACAD1, MCAD, MCADH, acyl-CoA dehydrogenase medium chain. ... acyl-CoA dehydrogenase activity. • identical protein binding. • oxidoreductase activity. • medium-chain-acyl-CoA dehydrogenase ... medium-chain fatty acid metabolic process. • Lipid metabolism. • fatty acid beta-oxidation using acyl-CoA dehydrogenase. • ...

https://en.wikipedia.org/wiki/ACADM

... is broken down into shorter-chain fatty acids in the human liver by the long-chain acyl CoA dehydrogenase enzyme. ... although the long-term use of Lorenzo's oil (oleic acid and erucic acid) in the treatment of adrenoleukodystrophy or ... While there are reports of toxicity from long-term use of Lorenzo's oil (which contains erucic acid and other ingredients), ... Erucic acid is produced by elongation of oleic acid via oleoyl-coenzyme A and malonyl-CoA. ...

https://en.wikipedia.org/wiki/Erucic_acid

If the fatty acyl-CoA has a long chain, then the carnitine shuttle must be utilized: Acyl-CoA is transferred to the hydroxyl ... is not an appropriate substrate for acyl CoA dehydrogenase, or enoyl CoA hydratase: If the acyl CoA contains a cis-Δ3 bond, ... Cn-acyl-CoA + FAD + NAD+ + H 2O + CoA → Cn-2-acyl-CoA + FADH 2 + NADH + H+ + acetyl-CoA Free fatty acids cannot penetrate any ... The final cycle produces two separate acetyl CoAs, instead of one acyl CoA and one acetyl CoA. For every cycle, the Acyl CoA ...

https://en.wikipedia.org/wiki/Beta_oxidation

CoA) hydratase, long-chain 3-hydroxy acyl-coenzyme A dehydrogenase and long-chain 3-ketoacyl CoA thiolase. Fatty acid beta- ... "Long-Chain Acyl CoA Dehydrogenase Deficiency: eMedicine Pediatrics: Genetics and Metabolic Disease". Retrieved 2009-07-11. Wang ...

https://en.wikipedia.org/wiki/Mitochondrial_trifunctional_protein

... deficiency of Acyl-CoA dehydrogenase, short chain, deficiency of Acyl-CoA dehydrogenase, very long chain, deficiency of Acyl- ... promyelocytic leukemia Acute renal failure Acute respiratory distress syndrome Acute tubular necrosis Acyl-CoA dehydrogenase, ... CoA oxidase deficiency Adactylia unilateral dominant ADAM complex Adams-Nance syndrome Adams-Oliver syndrome Addison's disease ... vitiligo Alpers disease Alpha 1-antitrypsin deficiency Alpha-2 deficient collagen disease Alpha-ketoglutarate dehydrogenase ...

https://en.wikipedia.org/wiki/List_of_diseases_(A)

Trimethoprim Triple A syndrome Tumors Tyrosinaemia type 1 Urea cycle disorder Uremia Very-long-chain acyl-CoA dehydrogenase ... Reye syndrome Ritonavir Saquinavir Sepsis Septic shock Severe hepatitis Sheehan syndrome Short-chain acyl-CoA dehydrogenase ... deficiency Maple syrup urine disease Mcquarrie type infantile idiopathic hypoglycemia Medium chain acyl-CoA dehydrogenase ... Disorders of fatty acid oxidation Medium chain acylCoA dehydrogenase deficiency (MCAD) Familial Leucine sensitive hypoglycemia ...

https://en.wikipedia.org/wiki/List_of_causes_of_hypoglycemia

... displays decreased thermal stability and is overrepresented in very-long-chain acyl-CoA dehydrogenase-deficient patients with ... A crystal structure of the complex of one of its interactors, medium-chain acyl-CoA dehydrogenase (MCAD; gene name ACADM) has ... "Acyl-CoA dehydrogenases, electron transfer flavoprotein and electron transfer flavoprotein dehydrogenase". Biochemical Society ... Crane FL, Beinert H (September 1954). "A Link Between Fatty Acyl CoA Dehydrogenase and Cytochrome C: A New Flavin Enzyme". ...

https://en.wikipedia.org/wiki/ETFA

"Very long-chain fatty acids". X-ald Database. Retrieved 5 January 2013. "Very long-chain acyl-CoA dehydrogenase deficiency". ... A very-long-chain fatty acid (VLCFA) is a fatty acid with 22 or more carbons. Their biosynthesis occurs in the endoplasmic ... doi:10.1016/j.plipres.2006.01.004 "Very-long-chain fatty acids from the animal and plant kingdoms" Rezanka, Tomas Progress in ... Trenkamp, S.; Martin, W.; Tietjen, K. (10 August 2004). "Specific and differential inhibition of very-long-chain fatty acid ...

https://en.wikipedia.org/wiki/Very_long_chain_fatty_acid

... or long-chain fatty acid acyl-CoA substrates. While different dehydrogenases target fatty acids of varying chain length, all ... "Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase: study of the effect of genetic ... "Mechanism of activation of acyl-CoA substrates by medium chain acyl-CoA dehydrogenase: interaction of the thioester carbonyl ... Acyl CoA Beta oxidation Thorpe, C.; Kim, J. J. (June 1995). "Structure and Mechanism of Action of the Acyl-CoA Dehydrogenases ...

https://en.wikipedia.org/wiki/Acyl-CoA_dehydrogenase

... as opposed to long-chain acyl-CoA synthetases, which ligate fatty acids to CoA, to produce the CoA ester. The role of the ACOT ... In the mitochondria, acyl-CoA esters are involved in the acylation of mitochondrial NAD+ dependent dehydrogenases; because ... Acyl-CoA thioesterase 9 is a protein that is encoded by the human ACOT9 gene. It is a member of the acyl-CoA thioesterase ... These enzymes have also been referred to in the literature as acyl-CoA hydrolases, acyl-CoA thioester hydrolases, and palmitoyl ...

https://en.wikipedia.org/wiki/Acyl-CoA_thioesterase_9

I The mechanism of elongation of long-chain fatty acids by acetyl-CoA". Biochim. Biophys. Acta. 164 (3): 498-517. doi:10.1016/ ... In enzymology, an acyl-CoA dehydrogenase (NADP+) (EC 1.3.1.8) is an enzyme that catalyzes the chemical reaction acyl-CoA + ... crotonyl-CoA reductase, and acyl-CoA dehydrogenase (NADP+). As of late 2007, only one structure has been solved for this class ... Other names in common use include 2-enoyl-CoA reductase, dehydrogenase, acyl coenzyme A (nicotinamide adenine dinucleotide, ...

https://en.wikipedia.org/wiki/Acyl-CoA_dehydrogenase_(NADP )

"Medium-Chain Acyl-CoA Dehydrogenase Deficiency". Medscape.. *^ Beermann, C.; Jelinek, J.; Reinecker, T.; Hauenschild, A.; Boehm ... In chemistry, particularly in biochemistry, a fatty acid is a carboxylic acid with a long aliphatic chain, which is either ... Malonyl-CoA is then involved in a repeating series of reactions that lengthens the growing fatty acid chain by two carbons at a ... The cytosolic acetyl-CoA is carboxylated by acetyl CoA carboxylase into malonyl-CoA, the first committed step in the synthesis ...

https://en.m.wikipedia.org/wiki/Straight-chain_fatty_acid

... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... Acyl-CoA dehydrogenase Medium-chain acyl-CoA dehydrogenase Butyryl-CoA (also known as butanoyl-CoA) Mahler HR (January 1954). " ... Short-chain acyl-CoA dehydrogenase (EC 1.3.8.1, butyryl-CoA dehydrogenase, butanoyl-CoA dehydrogenase, butyryl dehydrogenase, ... short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl CoA reductase, butanoyl-CoA:( ...

https://en.wikipedia.org/wiki/Short-chain_acyl-CoA_dehydrogenase

... (EC 1.3.8.8, palmitoyl-CoA dehydrogenase, palmitoyl-coenzyme A dehydrogenase, long-chain acyl ... long-chain-acyl-CoA:(acceptor) 2,3-oxidoreductase, ACADL (gene).) is an enzyme with systematic name long-chain acyl-CoA: ... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... Long-chain+acyl-CoA+dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) Biology portal. ...

https://en.wikipedia.org/wiki/Long-chain_acyl-CoA_dehydrogenase

... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... acyl dehydrogenase (ambiguous), fatty-acyl-CoA dehydrogenase (ambiguous), acyl CoA dehydrogenase (ambiguous), general acyl CoA ... Medium-chain acyl-CoA dehydrogenase (EC 1.3.8.7, fatty acyl coenzyme A dehydrogenase (ambiguous), acyl coenzyme A dehydrogenase ... dehydrogenase (ambiguous), medium-chain acyl-coenzyme A dehydrogenase, acyl-CoA:(acceptor) 2,3-oxidoreductase (ambiguous), ...

https://en.wikipedia.org/wiki/Medium-chain_acyl-CoA_dehydrogenase

... (EC 1.3.8.9, ACADVL (gene).) is an enzyme with systematic name very-long-chain acyl-CoA: ... "Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase ... Very-long-chain+acyl-CoA+dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) Biology portal. ... I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase". The Journal of Biological Chemistry. 267 (2 ...

https://en.wikipedia.org/wiki/Very-long-chain_acyl-CoA_dehydrogenase

These enzymes are better equipped to oxidize Acyl-CoA with long chains that the mitochondria cannot handle. Beta oxidation ... Beta oxidation of acyl-CoA occurs in four steps. 1. Acyl-CoA dehydrogenase catalyzes dehydrogenation of the acyl-CoA, creating ... The latter conversion is mediated by acyl-CoA synthase" acyl-P + HS-CoA → acyl-S-CoA + Pi + H+ Three types of acyl-CoA ... For example, the substrates for medium chain acyl-CoA synthase are 4-11 carbon fatty acids. The enzyme acyl-CoA thioesterase ...

https://en.wikipedia.org/wiki/Acyl-CoA

Roth, Karl S. (2013-12-19). "Medium-Chain Acyl-CoA Dehydrogenase Deficiency". Medscape. Beermann, C.; Jelinek, J.; Reinecker, T ... Long-chain fatty acids (LCFA) are fatty acids with aliphatic tails of 13 to 21 carbons. Very long chain fatty acids (VLCFA) are ... The cytosolic acetyl-CoA is carboxylated by acetyl CoA carboxylase into malonyl-CoA, the first committed step in the synthesis ... Hauenschild, A.; Boehm, G.; Klör, H.-U. (2003). "Short term effects of dietary medium-chain fatty acids and n−3 long-chain ...

https://en.wikipedia.org/wiki/Fatty_acid

Due to this mutation, effective levels of very long-chain-acyl-CoA-dehydrogenase are low or absent in the body, giving rise to ... A change of the gene that codes for very long-chain-acyl-CoA-dehydrogenase (VLCAD) results in a deficiency or malfunction of ... Mutations in the ACADVL gene lead to inadequate levels of an enzyme called very long-chain acyl-coenzyme A (CoA) dehydrogenase ... Very Long-Chain Acyl-CoA Dehydrogenase (VLCAD) Deficiency Information for Healthcare Professionals" (PDF). Kansas Department of ...

https://en.wikipedia.org/wiki/Very_long-chain_acyl-coenzyme_A_dehydrogenase_deficiency

... the fatty acyl-CoA dehydrogenases for short, medium, long, and very long acyl chains, and related enzymes. PPARα functions as a ... the fatty acyl group is transferred from fatty acyl-carnitine to coenzyme A, regenerating fatty acyl-CoA and a free carnitine ... displacing AMP to form thioester fatty acyl-CoA. In the second reaction, acyl-CoA is transiently attached to the hydroxyl group ... The first reaction of the carnitine shuttle is a two-step process catalyzed by a family of isozymes of acyl-CoA synthetase that ...

https://en.wikipedia.org/wiki/Carnitine

Dehydrogenase Deficiency (GAII & MADD) 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (HMG deficiency) Very long-chain acyl- ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (LCHAD deficiency) Medium-chain acyl-coenzyme A dehydrogenase ... Short-chain acyl-coenzyme A dehydrogenase deficiency (SCAD deficiency) 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (M/ ... 4 Dienoyl-CoA Reductase Deficiency Electron Transfer Flavoprotein (ETF) ...

https://en.wikipedia.org/wiki/Fatty-acid_metabolism_disorder

Short-chain hydroxy Acyl-CoA dehydrogenase deficiency (SCHAD) Long-chain acyl-CoA dehydrogenase deficiency (LCAD) Multiple acyl ... 1 in 75,000 Medium-chain acyl-CoA dehydrogenase deficiency (MCAD) > 1 in 25,000 Very-long-chain acyl-CoA dehydrogenase ... Medium/short-chain L-3-hydroxy acyl-CoA dehydrogenase deficiency Medium-chain ketoacyl-CoA thiolase deficiency Dienoyl-CoA ... 1 in 100,000 Inborn errors of fatty acid metabolism Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) > ...

https://en.wikipedia.org/wiki/List_of_disorders_included_in_newborn_screening_programs

... introduces a double bond into a fatty acyl side chain. TamH then carries out the reduction of the CoA-ester to form an aldehyde ... AfaA is hypothesized to activate long-chain fatty acids while the predicted dehydrogenase, TamT, ... The biosynthesis of tambjamine YP1 first involves the incorporation of proline, malonyl Co-A, and serine to form 4-methoxy-2,2 ... the majority have short alkyl chains connected to the enamine nitrogen. This group of alkaloids have been isolated from marine ...

https://en.wikipedia.org/wiki/Tambjamine

3-hydroxyacyl-CoA dehydrogenase activity. • RNA binding. • acetyl-CoA C-acyltransferase activity. • long-chain-enoyl-CoA ... transferring acyl groups other than amino-acyl groups. • enoyl-CoA hydratase activity. • long-chain-3-hydroxyacyl-CoA ... "The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-oxoacyl-CoA ... long-chain enoyl-CoA hydratase, and long-chain thiolase. This deficiency can be classified into 3 main clinical phenotypes: ...

https://en.wikipedia.org/wiki/HADHB

"Role of long-chain fatty acyl-CoA esters in the regulation of metabolism and in cell signalling". 》The Biochemical Journal》 323 ... 효소의 이름은 보통 효소의 기질이나 효소가 촉매하는 화학 반응에서 유래된 단어 끝에 접미사 "-에이스(-ase)"를 붙여서 짓는다.[2] 락테이스(lactase), 알코올 탈수소효소(alcohol dehydrogenase), ... Anfinsen CB (July 1973). "Principles that govern the folding of protein chains". 》Science》 181 (4096): 223-30. Bibcode:1973Sci ... "The discovery and development of HMG-CoA reductase inhibitors" (PDF). 》J. Lipid Res.》 33 (11): 1569-82. PMID 1464741 ...

https://ko.wikipedia.org/wiki/효소

Fatty acyl CoA dehydrogenase requires FAD in fatty acid oxidation. *FAD is required to convert retinol (vitamin A) to retinoic ... and branched-chain amino acids requires FAD in the shared E3 portion of their respective dehydrogenase complexes ... Long term vitamin status and dietary intake of health elderly subjects. I. Riboflavin. British J. of Nutr., 1979; 42:33-42 ... Flavoproteins of electron transport chain, including FMN in Complex I and FAD in Complex II ...

https://en.m.wikipedia.org/wiki/Riboflavin

isovaleryl-CoA dehydrogenase Ja 1.3.99.13 long-chain-acyl-CoA dehydrogenase Ja ... oxoglutarate dehydrogenase (succinyl-transferring) Ja 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl- ... L-iditol 2-dehydrogenase Ja 1.1.1.15 D-iditol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. D-sorbose + NADH + H+ D-iditol 2- ... D-arabitol 4-dehydrogenase 1.1.1.12 L-arabitol + NAD+ ⇌. {\displaystyle \rightleftharpoons }. L-xylulose + NADH + H+ L-arabitol ...

https://nl.wikipedia.org/wiki/Oxidoreductase

... particularly a fatty-acid oxidation disorder medium-chain acyl-CoA dehydrogenase deficiency.[11] ... 1/3rd long term disability[2][3]. Frequency. Less than one in a million children a year[2]. ...

https://en.wikipedia.org/wiki/Reye's_syndrome

Carnitine palmitoyltransferase II deficiency (also known as CPT-II deficiency) leads to an excess long chain fatty acids, as ... and the regulation of pyruvate dehydrogenase (PDH), which converts pyruvate to acetyl CoA. Transferases are also utilized ... Transfer of acyl groups or acyl groups that become alkyl groups during the process of being transferred are key aspects of EC ... Succinyl-CoA:3-ketoacid CoA transferase deficiency (or SCOT deficiency) leads to a buildup of ketones. Ketones are created upon ...

https://en.wikipedia.org/wiki/Transferase

to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... This conversion begins with the negatively charged carboxylate side chain oxygen atom of Asp-375 deprotonating acetyl CoA's ... transferase activity, transferring acyl groups, acyl groups converted into alkyl on transfer. • citrate (Si)-synthase activity ... The enzyme is inhibited by high ratios of ATP:ADP, acetyl-CoA:CoA, and NADH:NAD, as high concentrations of ATP, acetyl-CoA, and ...

https://en.wikipedia.org/wiki/Citrate_synthase

"Role of long-chain fatty acyl-CoA esters in the regulation of metabolism and in cell signalling". The Biochemical Journal. 323 ... Other allosteric activators include glutamate and other dicarboxylic acids.[16] Long and short chain fatty acyl CoAs are ... is produced which is a building block for new fatty acids and can inhibit the transfer of the fatty acyl group from acyl CoA to ... Biotin is covalently attached through an amide bond to the long side chain of a lysine reside in BB. As BB is between BC and CT ...

https://en.wikipedia.org/wiki/Acetyl-CoA_carboxylase

Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase". J. Biol. Chem. 258 (2): 1066-76. PMID 6401712. ... Electrons move quite long distances through proteins by hopping along chains of these cofactors.[13] This occurs by quantum ... "Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. ... Eukaryotic electron transport chainsEdit. Further information: Electron transport chain and Chemiosmosis ...

https://en.m.wikipedia.org/wiki/Oxidative_phosphorylation

Hydroxyacyl-Coenzyme A dehydrogenase. To acetyl-CoA. *Malonyl-CoA decarboxylase. Aldehydes. *Long-chain-aldehyde dehydrogenase ... 3R)-3-hydroxyacyl-[acyl-carrier-protein] + NADP+ ⇌. {\displaystyle \rightleftharpoons }. 3-oxoacyl-[acyl-carrier-protein] + ... Other names in common use include beta-ketoacyl-[acyl-carrier protein](ACP) reductase, beta-ketoacyl acyl carrier protein (ACP ... In enzymology, a 3-oxoacyl-[acyl-carrier-protein] reductase (EC 1.1.1.100) is an enzyme that catalyzes the chemical reaction ...

https://en.wikipedia.org/wiki/Beta-Ketoacyl_ACP_reductase

3-Hydroxybutyryl-CoA dehydrogenase. *Branched-chain amino acid aminotransferase. *Branched-chain alpha-keto acid dehydrogenase ... Glutamine is formed if an ammonium ion attacks the acyl-phosphate intermediate, while glutamate is remade if water attacks the ... Acetyl-CoA synthetase. *Long-chain-fatty-acid-CoA ligase. 6.3: Carbon-Nitrogen. *Glutamine synthetase ... Methylglutaconyl-CoA hydratase. (See Template:Leucine metabolism in humans - this diagram does not include the pathway for β- ...

https://en.wikipedia.org/wiki/Glutamine_synthetase

Dozens of ATP equivalents are generated by the beta-oxidation of a single long acyl chain.[20] The acetyl-CoA produced by beta- ... In the mitochondrion, pyruvate is oxidized by the pyruvate dehydrogenase complex to the acetyl group, which is fully oxidized ... In the presence of air and various cofactors and enzymes, fatty acids are degraded to acetyl-CoA. The pathway is called beta- ... Two equivalents of NADH are also produced, which can be oxidized via the electron transport chain and result in the generation ...

https://en.m.wikipedia.org/wiki/Adenosine_triphosphate

As a result, the food will have a longer shelf life (part of the reason foods are purposely fermented in the first place); ... Glycolysis → Pyruvate decarboxylation → Citric acid cycle → Oxidative phosphorylation (electron transport chain + ATP synthase) ... The reaction is catalysed by the enzymes pyruvate decarboxylase and alcohol dehydrogenase.[13] ... Acyl-CoA Fatty. acids Glyco-. sphingolipids Sphingolipids Waxes Polyunsaturated. fatty acids Neurotransmitters. & thyroid ...

https://en.wikipedia.org/wiki/Fermentation

It can fit into hydrophilic or hydrophobic environments, due to its minimal side chain of only one hydrogen atom. The acyl ... In higher eukaryotes, δ-aminolevulinic acid, the key precursor to porphyrins, is biosynthesized from glycine and succinyl-CoA ... Glyoxylate is then oxidized by hepatic lactate dehydrogenase to oxalate in an NAD+-dependent reaction.[26] ... CS1: long volume value. *CS1 French-language sources (fr). *CS1 maint: BOT: original-url status unknown ...

https://en.wikipedia.org/wiki/Glycine

A covalent bond formed between the active site and DIFP, so the serine side chain is no longer available to the substrate.[17] ... ATP-dependent carboxylation of acetyl-CoA to malonyl-CoA. Lipids are important in making up the cell membrane. ... For example, alcohol dehydrogenase which catalyses the transfer of a hydride ion from ethanol to NAD+ interacts with the ... The resulting hydroxide anion nucleophilically attacks the acyl-enzyme complex to form a second tetrahedral oxyanion ...

https://en.m.wikipedia.org/wiki/Active_site

Long-chain-fatty-acid-CoA ligase. tryptophan metabolism. *Kynureninase. monoamine neurotransmitter. metabolism. *Monoamine ... Enoyl-acyl carrier protein reductase/Enoyl ACP reductase. *7-Dehydrocholesterol reductase. *Biliverdin reductase ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ...

https://en.wikipedia.org/wiki/Succinate_dehydrogenase

3-Hydroxybutyryl-CoA dehydrogenase. *Branched-chain amino acid aminotransferase. *Branched-chain alpha-keto acid dehydrogenase ... This is followed by Cβ-Cγ bond cleavage to generate an acyl-enzyme intermediate together with a tautomerized Ala-PLP adduct. ... Methylglutaconyl-CoA hydratase. (See Template:Leucine metabolism in humans - this diagram does not include the pathway for β- ... tryptophan catabolic process to acetyl-CoA. • L-kynurenine catabolic process. • tryptophan catabolic process. • tryptophan ...

https://en.wikipedia.org/wiki/Kynureninase

Beta oxidation, in the mitochondrial matrix, then cuts the long carbon chains of the fatty acids (in the form of acyl-CoA ... Dehydrogenation by acyl-CoA dehydrogenase, yielding 1 FADH2 Hydration by enoyl-CoA hydratase Dehydrogenation by 3-hydroxyacyl- ... In order for the acyl-CoA to enter the mitochondrion the carnitine shuttle is used: Acyl-CoA is transferred to the hydroxyl ... Acetyl-CoA is formed into malonyl-CoA by acetyl-CoA carboxylase, at which point malonyl-CoA is destined to feed into the fatty ...

https://en.wikipedia.org/wiki/Fatty_acid_metabolism

The histidine-bound heme group of succinate dehydrogenase, an electron carrier in the mitochondrial electron transfer chain. ... Initially thought essential only for infants, longer-term studies have shown it is essential for adults also.[3] It is encoded ... The acyl radical is histidyl. Contents. *1 Chemical properties *1.1 NMR and tautomerism ... G→propionyl-CoA→. succinyl-CoA. valine→. *α-Ketoisovaleric acid. *Isobutyryl-CoA. *Methacrylyl-CoA ...

https://en.wikipedia.org/wiki/L-Histidine

Acyl CoA dehydrogenase. *Apoptosis-inducing factor. *Butyryl CoA dehydrogenase. *Cryptochrome. *Cytochrome b5 reductase ... of the N2 cluster and the relative proximity of the other clusters in the chain enable efficient electron transfer over long ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial. Pfam PF01370 19. NDUFAB1 / ACPM. ACPM_HUMAN. Acyl ...

https://en.wikipedia.org/wiki/NADH_dehydrogenase_

Dihydroorotate dehydrogenase. *Coproporphyrinogen III oxidase. *Protoporphyrinogen oxidase. *Bilirubin oxidase. *Acyl-CoA ... mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone). • mitochondrial inner membrane. • ... occurs and the SDHB protein is no longer produced. Tumor formation then becomes possible. ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ...

https://en.wikipedia.org/wiki/SDHB

acyl groups. Bacteria, archaea and eukaryotes Cobalamine [5]. Cobalamine (B12). None. hydrogen, alkyl groups. Bacteria, archaea ... Through a long and difficult purification from yeast extracts, this heat-stable factor was identified as a nucleotide sugar ... For example, the multienzyme complex pyruvate dehydrogenase[6] at the junction of glycolysis and the citric acid cycle requires ... Denessiouk KA, Rantanen VV, Johnson MS (August 2001). "Adenine recognition: a motif present in ATP-, CoA-, NAD-, NADP-, and FAD ...

https://en.wikipedia.org/wiki/Cofactor_

... in a newborn due to medium chain acyl CoA dehydrogenase (MCAD) deficiency with an unusual severe genotype» (en anglès). Ital J ... Jung SY, Lee YJ, Lee HJ, Lee YA, et al «Nonalcoholic fatty liver disease in long-term survivors of childhood-onset ... Dalvi PS, Yang S, Swain N, Kim J, et al «Long-term metabolic effects of malnutrition: Liver steatosis and insulin resistance ... acetil-CoA carboxilasa, el GS-0976, disminueix significativament la quantitat de greix hepàtic dels malalts d'esteatohepatitis ...

https://ca.m.wikipedia.org/wiki/Esteatosi_hepàtica

EC 6.2.1.2: Medium-chain acyl-CoA ligase. *EC 6.2.1.3: Long-chain-fatty-acid--CoA ligase ... Glycerol dehydrogenase EC 1.1.1.6. *Propanediol-phosphate dehydrogenase EC 1.1.1.7. *glycerol-3-phosphate dehydrogenase (NAD+) ... EC 6.2.1.40: 4-hydroxybutyrate--CoA ligase. *EC 6.2.1.41: 3-((3aS,4S,7aS)-7a-methyl-1,5-dioxo-octahydro-1H-inden-4-yl) ... EC 6.2.1.38: (2,2,3-trimethyl-5-oxocyclopent-3-enyl)acetyl-CoA synthase ...

https://en.m.wikipedia.org/wiki/List_of_enzymes

ACADM: acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain ... "p": Short arm; "q": Long arm. *^ For cytogenetic banding ... 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. *Hypertrophic cardiomyopathy, autosomal dominant mutations of TNNT2; ... "Long-term follow-up of R403WMHY7 and R92WTNNT2 HCM families: mutations determine left ventricular dimensions but not wall ...

https://en.m.wikipedia.org/wiki/Chromosome_1

Hydroxyacyl-Coenzyme A dehydrogenase. To acetyl-CoA. *Malonyl-CoA decarboxylase. Aldehydes. *Long-chain-aldehyde dehydrogenase ... Enoyl-acyl carrier protein reductase (or ENR) (EC 1.3.1.9), is a key enzyme of the type II fatty acid synthesis (FAS) system.[1 ... "Identification and characterization of inhibitors of bacterial enoyl-acyl carrier protein reductase". Antimicrobial Agents and ... "Mutational analysis of the triclosan-binding region of enoyl-ACP (acyl-carrier protein) reductase from Plasmodium falciparum" ...

https://en.wikipedia.org/wiki/Enoyl-acyl_carrier_protein_reductase

Fatty acyl CoA dehydrogenase requires FAD in fatty acid oxidation. *FAD is required to convert retinol (vitamin A) to retinoic ... and branched-chain amino acids requires FAD in the shared E3 portion of their respective dehydrogenase complexes ... It provides a measure of tissue saturation and long-term riboflavin status. In vitro enzyme activity in terms of activity ... Flavoproteins of electron transport chain, including FMN in Complex I and FAD in Complex II ...

https://en.m.wikipedia.org/wiki/Vitamin_B2

Examples of this type of disorder are Albinism, Medium-chain acyl-CoA dehydrogenase deficiency, cystic fibrosis, sickle-cell ... Long QT syndrome 10. *SCN5A *Brugada syndrome 1. *Long QT syndrome 3 ...

https://en.wikipedia.org/wiki/Genetic_defects

Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a condition that prevents the body from converting certain fats to ... medlineplus.gov/genetics/condition/very-long-chain-acyl-coa-dehydrogenase-deficiency/ Very long-chain acyl-CoA dehydrogenase ... Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a condition that prevents the body from converting certain fats to ... This gene provides instructions for making an enzyme called very long-chain acyl-CoA dehydrogenase, which is required to break ...

https://medlineplus.gov/genetics/condition/very-long-chain-acyl-coa-dehydrogenase-deficiency/

Long-chain acyl-CoA dehydrogenase (EC 1.3.8.8, palmitoyl-CoA dehydrogenase, palmitoyl-coenzyme A dehydrogenase, long-chain acyl ... long-chain-acyl-CoA:(acceptor) 2,3-oxidoreductase, ACADL (gene).) is an enzyme with systematic name long-chain acyl-CoA: ... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... Long-chain+acyl-CoA+dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) Biology portal. ...

https://en.wikipedia.org/wiki/Long-chain_acyl-CoA_dehydrogenase

Very-long-chain acyl-CoA dehydrogenase (EC 1.3.8.9, ACADVL (gene).) is an enzyme with systematic name very-long-chain acyl-CoA: ... "Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase ... Very-long-chain+acyl-CoA+dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) Biology portal. ... I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase". The Journal of Biological Chemistry. 267 (2 ...

https://en.wikipedia.org/wiki/Very-long-chain_acyl-CoA_dehydrogenase

ACADVL acyl-CoA dehydrogenase very long chain [Homo sapiens] ACADVL acyl-CoA dehydrogenase very long chain [Homo sapiens]. Gene ... Very long chain acyl-CoA dehydrogenase. * NM_001033859.3 → NP_001029031.1 very long-chain specific acyl-CoA dehydrogenase, ... Very long chain acyl-CoA dehydrogenase. * XM_011523830.2 → XP_011522132.1 very long-chain specific acyl-CoA dehydrogenase, ... very long-chain specific acyl-CoA dehydrogenase, mitochondrial. Names. acyl-Coenzyme A dehydrogenase, very long chain. NP_ ...

https://www.ncbi.nlm.nih.gov/gene/?term=37

2 patients with very long chain Acyl-CoA dehydrogenase deficiency experience fatigue, insomnia, depressed mood, pain, and ... Find the most comprehensive real-world symptom and treatment data on very long chain Acyl-CoA dehydrogenase deficiency at ... What is very long chain Acyl-CoA dehydrogenase deficiency?. Very long chain acyl-coenzyme A dehydrogenase deficiency is a ... Who has very long chain Acyl-CoA dehydrogenase deficiency on PatientsLikeMe?. * 2 patients have this condition ...

https://www.patientslikeme.com/conditions/2525-very-long-chain-acyl-coa-dehydrogenase-deficiency

Very long-chain-specific acyl-CoA dehydrogenase, mitochondrialImported. ,p>Information which has been imported from another ... tr,J3QKU9,J3QKU9_HUMAN Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial (Fragment) OS=Homo sapiens OX=9606 GN= ... Very long-chain specific acyl-CoA dehydrogenase, mitochondrial, VLCAD, EC 1.3.8.9 ... Very long-chain-specific acyl-CoA d.... Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial ...

https://www.uniprot.org/uniprot/J3QKU9

PF00441. Acyl-CoA_dh_1. 1 hit. PF02770. Acyl-CoA_dh_M. 1 hit. PF02771. Acyl-CoA_dh_N. 1 hit. ... PF00441. Acyl-CoA_dh_1. 1 hit. PF02770. Acyl-CoA_dh_M. 1 hit. PF02771. Acyl-CoA_dh_N. 1 hit. ... Very long-chain-specific acyl-CoA dehydrogenase, mitochondrialImported. ,p>Information which has been imported from another ... tr,B1AR28,B1AR28_MOUSE Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial OS=Mus musculus GN=Acadvl PE=1 SV=1 ...

http://www.uniprot.org/uniprot/B1AR28

Species about Experts and Doctors on long chain acyl coa dehydrogenase in Düsseldorf, North Rhine Westphalia, Germany ... long chain acyl coa dehydrogenase*inborn errors lipid metabolism*dihydroxyacetone phosphate*fructosephosphates*acyl coa ... Experts and Doctors on long chain acyl coa dehydrogenase in Düsseldorf, North Rhine Westphalia, Germany. Summary. Locale: ... You are here: Locale , Germany , North Rhine Westphalia , Experts and Doctors on long chain acyl coa dehydrogenase in ...

http://www.labome.org/locale/germany/north/experts-and-doctors-on-long-chain-acyl-coa-dehydrogenase-in-d--sseldorf--north-rhine-westphalia--germany-2056993.html

Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the initial rate-limiting step in mitochondrial fatty acid beta- ... Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency Am J Hum Genet. 1999 ... Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the initial rate-limiting step in mitochondrial fatty acid beta- ... clear genotype-phenotype relationship is in sharp contrast to what has been observed in medium-chain acyl-CoA dehydrogenase ...

https://pubmed.ncbi.nlm.nih.gov/9973285/?dopt=Abstract

Rhabdomyolysis is common in very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) and other metabolic myopathies, but its ...

https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0147818

"Acyl-CoA Dehydrogenase, Long-Chain" by people in this website by year, and whether "Acyl-CoA Dehydrogenase, Long-Chain" was a ... Acyl-CoA Dehydrogenase, Long-Chain*Acyl-CoA Dehydrogenase, Long-Chain. *Acyl CoA Dehydrogenase, Long Chain ... Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy. Mol Ther. 2012 Jun; ... Long-Chain Acyl-CoA Dehydrogenase. *Very-Long-Chain Acyl-CoA Dehydrogenase. *Acyl-CoA Dehydrogenase, Very-Long-Chain ...

https://profiles.umassmed.edu/display/123583

A severe genotype with favourable outcome in very long chain acyl-CoA dehydrogenase deficiency ... A severe genotype with favourable outcome in very long chain acyl-CoA dehydrogenase deficiency ...

https://adc.bmj.com/content/84/1/58.alerts

... very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase ... 1993) Very-long-chain acyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid ... Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a recently identified inborn error of a membrane bound ... 1993) A novel disease with deficiency of mitochondrial very-long-chain acyl-CoA dehydrogenase. Biochem Biophys Res Commun 191: ...

http://adc.bmj.com/content/84/1/58

Very Long Chain Acyl CoA Dehydrogenase Deficiency (VLCAD)Download version for offline viewing or printing. [480.71kB] ... Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare, inherited (genetic) disease. ... Treatment is started as early as possible and is usually life long. Babies with VLCAD have their health and development checked ... Babies with VLCAD cannot make certain fats into energy, especially during long periods without food (fasting). ...

https://www.newbornscreening.on.ca/en/disease/very-long-chain-acyl-coa-dehydrogenase-deficiency-vlcad

... long chain (ACADL), nuclear gene encoding mitochondrial protein, 200 uL, ,10^7 TU/mL, 200 µl. ... Home » ORF » Human Lenti ORF Particles » Lenti ORF particles, ACADL (mGFP-tagged)-Human acyl-CoA dehydrogenase, long chain ( ... RC206624L2V Lenti ORF particles, ACADL (mGFP-tagged)-Human acyl-CoA dehydrogenase, long chain (ACADL), nuclear gene encoding ... Properties for Lenti ORF particles, ACADL (mGFP-tagged)-Human acyl-CoA dehydrogenase, long chain (ACADL), nuclear gene encoding ...

https://www.acris-antibodies.com/orf/human-lenti-orf-particles/lenti-orf-particles-acadl-mgfp-tagged-human-acyl-coa-dehydrogenase-long-chain-acadl-nuclear-gene-encoding-mitochondrial-protein-200-ul-10-7-tu-ml-rc206624l2v.htm

... Common Name(s). Very long chain acyl-CoA dehydrogenase deficiency, VCLAD ... "Very long chain acyl-CoA dehydrogenase deficiency" (open studies are recruiting volunteers) and 9 "Very long chain acyl-CoA ... Very Long-chain Acyl-CoA Dehydrogenase Deficiency; Trifunctional Protein Deficiency; Long-chain 3-hydroxyacyl-CoA Dehydrogenase ... Medium-chain Acyl-CoA Dehydrogenase Deficiency; Multiple Acyl-CoA Dehydrogenase Deficiency; Carnitine Transporter Deficiency; ...

http://diseaseinfosearch.org/ACADVL/7410

Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can ... This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product ... HCA RNA Cell Line for Very long-chain specific acyl-CoA dehydrogenase, mitochondrial. ... Compartment GO Terms for Very long-chain specific acyl-CoA dehydrogenase, mitochondrial. ...

https://pharos.nih.gov/idg/targets/P49748

Very long chain acyl-coA dehydrogenase deficiency (VLCADD) is an autosomal recessive inborn error of fatty acid oxidation ... Infants suspected to have very-long chain acyl-CoA dehydrogenase deficiency from newborn screening.. *J Lawrence Merritt, ... Positive newborn screen in a normal infant of a mother with asymptomatic very long-chain Acyl-CoA dehydrogenase deficiency.. * ... Very long chain acyl-coA dehydrogenase deficiency (VLCADD) is an autosomal recessive inborn error of fatty acid oxidation ...

https://www.semanticscholar.org/paper/Recurrent-ACADVL-molecular-findings-in-individuals-Miller-Burrage/5e68f40d49ef6b427b9f43472ff429a904115cbe

... is one of four straight-chain acyl-CoA dehydrogenase (ACD) enzymes, which are all nuclear encoded mitochondrial flavoproteins ... Moreover, human VLCAD and human acyl-CoA oxidase showed extensive sequence homology corroborating the notion that these genes ... Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of four straight-chain acyl-CoA dehydrogenase (ACD) enzymes, which are ... Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency.. Manuel Schiff, A Mohsen, +3 authors ...

https://www.semanticscholar.org/paper/Cloning-and-characterization-of-human-acyl-CoA-of-Andresen-Bross/605cd0448bf4dd01cecb543db41e596b7e9d7fe2

... Critical elevation of C14:1 acylcarnitine.. What does this mean? ... InicioAbnormal Newborn ScreeningFor Healthcare ProvidersFatty Acid Oxidation DisordersVery long chain acyl CoA dehydrogenase ... This infant may have Very Long Chain Acyl-oA Dehydrogenase deficiency. Further testing is required. ... Medium chain acyl CoA dehydrogenase deficiency (MCAD). *Trifunctional protein deficiency (TFP). *Very long chain acyl CoA ...

https://es.archildrens.org/abnormal-newborn-screening/for-healthcare-providers/fatty-acid-oxidation-disorders/very-long-chain-acyl-coa-dehydrogenase-deficiency-

The ACADVL gene provides instructions for making an enzyme called very long-chain acyl-CoA dehydrogenase (VLCAD). Learn about ... Very long-chain acyl-CoA dehydrogenase is required to metabolize a group of fats called very long-chain fatty acids. These ... Very long-chain acyl-CoA dehydrogenase deficiency. More than 100 mutations in the ACADVL gene have been found to cause very ... Very long-chain acyl-CoA dehydrogenase is essential for fatty acid oxidation, which is the multistep process that breaks down ( ...

https://medlineplus.gov/genetics/gene/acadvl/

Long-chain-acyl-CoA dehydrogenase (LCADH) has been produced by recombinant techniques from the human cDNA and purified after ... Characterization of human and pig kidney long-chain-acyl-CoA dehydrogenases and their role in beta-oxidation. * Home ... Characterization of human and pig kidney long-chain-acyl-CoA dehydrogenases and their role in beta-oxidation. Publikationstyp: ... Characterization_of_human_and_pig_kidney_long_chain_acyl_CoA_dehydrogenases_and_their_role_in_beta_oxidation.pdf. 142. ...

https://kops.uni-konstanz.de/handle/123456789/7772

... which is associated with very long chain acyl-CoA dehydrogenase (VLCAD) deficiency and elevated C14, C14:1 acylcarnitines on ... Invitae Very Long Chain Acyl-CoA Dehydrogenase Deficiency Test. Test description. The Invitae Very Long Chain Acyl-CoA ... Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency. Am. J. Hum. Genet. ... Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency. Am. J. Hum. Genet. ...

https://www.invitae.com/en/physician/tests/06167/?cat=CAT000045

Very long-chain acyl CoA dehydrogenase deficiency which was accepted as infanticide ...

https://avesis.gazi.edu.tr/yayin/64e623bd-a63f-4ffa-be33-14abb3771be0/very-long-chain-acyl-coa-dehydrogenase-deficiency-which-was-accepted-as-infanticide

... very long chain, deficiency of? Medigest has all you need to know about Acyl-CoA dehydrogenase, very long chain, deficiency of ... Discuss Acyl-CoA dehydrogenase, very long chain, deficiency of in our forums Discuss Acyl-CoA dehydrogenase, very long chain, ... Acyl-CoA dehydrogenase, very long chain, deficiency of Below you will find more information about Acyl-CoA dehydrogenase, very ... Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) is a rare autosomal recessive condition in which the body fails to ...

https://www.medigest.uk/diseases/acyl-coa-dehydrogenase-very-long-chain-deficiency-of/

Very long-chain acyl-CoA dehydrogenase is required to metabolize a group of fats called very long-chain fatty acids. These ... acyl-CoA dehydrogenase very long chain. Enable Javascript to view the expand/collapse boxes.. Printable PDF Open All Close All ... Very long-chain acyl-CoA dehydrogenase is essential for fatty acid oxidation, which is the multistep process that breaks down ( ... More than 100 mutations in the ACADVL gene have been found to cause very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency. ...

https://ghr.nlm.nih.gov/gene/ACADVL

Clinical and biochemical outcome of patients with very long-chain acyl-CoA dehydrogenase deficiency Valentina Rovelli 1 , ... Clinical and biochemical outcome of patients with very long-chain acyl-CoA dehydrogenase deficiency Valentina Rovelli et al. ... Background: Very-Long-Chain Acyl-CoA Dehydrogenase (VLCAD) deficiency is a disorder of fatty acid oxidation included in the ... Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. Schiff M, Mohsen AW, Karunanidhi A, ...

https://phgkb.cdc.gov/PHGKB/phgHome.action?action=forward&dbsource=scan&id=25774

Very-Long-Chain Acyl-CoA Dehydrogenase (VLCAD) deficiency is a disorder of fatty acid oxidation included in the recommended ... Clinical and biochemical outcome of patients with very long-chain acyl-CoA dehydrogenase deficiency.. Rovelli V1, Manzoni F2, ... BabysFirstTest - Very Long-Chain Acyl-CoA Dehydrogenase Deficiency - Genetic Alliance. *Lipid Metabolism Disorders - ... Acyl-CoA Dehydrogenase, Long-Chain/deficiency*. *Acyl-CoA Dehydrogenase, Long-Chain/genetics ...

https://phgkb.cdc.gov/PHGKB/phgHome.action?action=forward&dbsource=phg&id=25774

Medium-chain acyl-CoA dehydrogenase deficiency. Very long-chain acyl-CoA dehydrogenase deficiency ... a secondary marker for medium chain acyl-CoA dehydrogenase deficiency), immunoreactive trypsinogen (a primary marker for cystic ... Laboratories also may retain tested specimens for a longer period or indefinitely for quality assurance and educational ... Short-chain acyl-CoA dehydrogenase deficiency. Medium/short-chain L-3-hydroxyacyl-CoA ...

https://www.cdc.gov/mmwr/preview/mmwrhtml/rr6102a1.htm

Very long-chain acyl-CoA dehydrogenase deficiency. 69. Biotinidase deficiency (including partial) ... medium-chain acyl-CoA dehydrogenase deficiency (Table) (3.6). Newborn screening can help prevent death or disability, if ... Long-term follow-up. The goal of long-term follow-up is to improve the quality of care for children with diagnosed disorders so ... Long-term follow-up is used to assess the needs of patients and families regarding disease management, treatment, and age- ...

http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6121a2.htm

Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a condition that prevents the body from converting certain fats to energy, particularly during periods without food (fasting). (medlineplus.gov)
One potential hotspot ACADVL mutation in Chinese patients with very-long-chain acyl-coenzyme A dehydrogenase deficiency. (nih.gov)
Analysis of ACADVL gene variations among nine neonates with very long chain acyl-coA dehydrogenase deficiency]. (nih.gov)
The diagnostic challenge in very-long chain acyl-CoA dehydrogenase deficiency (VLCADD). (nih.gov)
Sequencing of the ACADVL gene revealed that all individuals with activities below 24% were true Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) patients, individuals with residual activities between 24 and 27% carried either one or two mutations. (nih.gov)
When you share what it's like to have very long chain Acyl-CoA dehydrogenase deficiency through your profile, those stories and data appear here too. (patientslikeme.com)
Got a question about living with very long chain Acyl-CoA dehydrogenase deficiency? (patientslikeme.com)
A novel tandem mass spectrometry method for rapid confirmation of medium- and very long-chain acyl-CoA dehydrogenase deficiency in newborns. (labome.org)
This clear genotype-phenotype relationship is in sharp contrast to what has been observed in medium-chain acyl-CoA dehydrogenase deficiency, in which no correlation between genotype and phenotype can be established. (nih.gov)
Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy. (umassmed.edu)
A near-miss: very long chain acyl-CoA dehydrogenase deficiency with normal primary markers in the initial well-timed newborn screening specimen. (umassmed.edu)
Spiekerkoetter U, Sun B, Zytkovicz T, Wanders R, Strauss AW, Wendel U. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency. (umassmed.edu)
A patient with very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is reported. (bmj.com)
Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a recently identified inborn error of a membrane bound mitochondrial enzyme. (bmj.com)
Electrospray tandem mass spectrometry (ESI-MSMS) of dried blood spots on filter paper taken three days after l -carnitine treatment identified increased long chain acylcarnitines suggestive of VLCAD deficiency. (bmj.com)
Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare, inherited (genetic) disease. (newbornscreening.on.ca)
Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare genetic condition resulting from a mutation (change) in a person's DNA. (diseaseinfosearch.org)
Following organizations serve the condition "Very long chain acyl-CoA dehydrogenase deficiency" for support, advocacy or research. (diseaseinfosearch.org)
Recurrent ACADVL molecular findings in individuals with a positive newborn screen for very long chain acyl-coA dehydrogenase (VLCAD) deficiency in the United States. (semanticscholar.org)
Very long chain acyl-coA dehydrogenase deficiency (VLCADD) is an autosomal recessive inborn error of fatty acid oxidation detected by newborn screening (NBS). (semanticscholar.org)
Infants suspected to have very-long chain acyl-CoA dehydrogenase deficiency from newborn screening. (semanticscholar.org)
Positive newborn screen in a normal infant of a mother with asymptomatic very long-chain Acyl-CoA dehydrogenase deficiency. (semanticscholar.org)
A heterozygous missense mutation in adolescent-onset very long-chain acyl-CoA dehydrogenase deficiency with exercise-induced rhabdomyolysis. (semanticscholar.org)
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency. (semanticscholar.org)
Successful Treatment of Cardiomyopathy due to Very Long-Chain Acyl-CoA Dehydrogenase Deficiency: First Case Report from Oman with Literature Review. (semanticscholar.org)
This infant may have Very Long Chain Acyl-oA Dehydrogenase deficiency. (archildrens.org)
More than 100 mutations in the ACADVL gene have been found to cause very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency. (medlineplus.gov)
With a shortage (deficiency) of functional VLCAD enzyme, very-long chain fatty acids are not metabolized properly. (medlineplus.gov)
Aoyama T, Souri M, Ushikubo S, Kamijo T, Yamaguchi S, Kelley RI, Rhead WJ, Uetake K, Tanaka K, Hashimoto T. Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients. (medlineplus.gov)
Pons R, Cavadini P, Baratta S, Invernizzi F, Lamantea E, Garavaglia B, Taroni F. Clinical and molecular heterogeneity in very-long-chain acyl-coenzyme A dehydrogenase deficiency. (medlineplus.gov)
The Invitae Very Long Chain Acyl-CoA Dehydrogenase Deficiency Test analyzes the ACADVL gene, which is associated with very long chain acyl-CoA dehydrogenase ( VLCAD ) deficiency. (invitae.com)
A low-fat, high-carbohydrate diet with medium-chain triglyceride ( MCT ) and avoidance of fasting has been used to treat patients with VLCAD deficiency. (invitae.com)
Below you will find more information about Acyl-CoA dehydrogenase, very long chain, deficiency of from Medigest. (medigest.uk)
If you believe that you are suffering from any of the symptoms of Acyl-CoA dehydrogenase, very long chain, deficiency of it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. (medigest.uk)
There are medical conditions that carry similar symptoms associated with Acyl-CoA dehydrogenase, very long chain, deficiency of and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. (medigest.uk)
The information relating to Acyl-CoA dehydrogenase, very long chain, deficiency of comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown. (medigest.uk)
Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) is a rare autosomal recessive condition in which the body fails to oxidize fatty acids because an enzyme is either missing or not functioning correctly. (medigest.uk)
Some patients who are deficient in all 3 enzymatic activities of the protein have been described, though most have an isolated LCHAD deficiency, which leads to the inability to metabolize long-chain fatty acids. (medigest.uk)
Very-Long-Chain Acyl-CoA Dehydrogenase (VLCAD) deficiency is a disorder of fatty acid oxidation included in the recommended uniform newborn screening (NBS) panel in the USA. (cdc.gov)
The five most commonly diagnosed conditions in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) cystic fibrosis, 4) sickle cell disease, and 5) medium-chain acyl-CoA dehydrogenase deficiency ( Table ) (3.6). (cdc.gov)
Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD, respectively) deficiency, using acylcarnitine profiling with tandem mass spectrometry, has increased the number of patients with fatty acid oxidation disorders due to the identification of additional milder, and so far silent, phenotypes. (cdc.gov)
To investigate the clinical and laboratory features of very long chain acyl-CoA dehydrogenase deficiency ( VLCADD ) and the correlations between its genotype and phenotype . (bvsalud.org)
Deficiency of very long-chain acyl-CoA dehydrogenase (VLCAD) is the most common disorder of mitochondrial β-oxidation of long-chain fatty acids. (springeropen.com)
[email protected]#To explore the clinical features and variations of ACADVL gene in 9 neonates with very long chain acyl-coenzyme A dehydrogenase deficiency (VLCADD). (bvsalud.org)
These included 2 cases with medium-chain acyl-CoA dehydrogenase deficiency, 4 cases consistent with glutaric acidemia type 2, 4 cases with either very long-chain acylcoenzyme A dehydrogenase deficiency or long-chain 3-hydroxy-acyl-coenzyme A dehydrogenase deficiency, and 4 cases predicted to be affected with carnitine uptake defect. (nih.gov)
A lactate/pyruvate ratio less than 25 suggests pyruvate dehydrogenase deficiency (PDH) or other disorders of pyruvate metabolism, whereas a ratio greater than 25 suggests a respiratory chain defect. (medscape.com)
Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. (wikibooks.org)
Very-long chain acyl-CoA dehydrogenase deficiency (VLCADD) is a clinically heterogeneous disorder with three major phenotypes: severe neonatal/infantile, milder childhood and late onset myopathic. (mdpi.com)
49 Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is a rare genetic condition that prevents the body from converting certain fats (called short-chain fatty acids) into energy. (malacards.org)
These mutations lead to a shortage (deficiency) of an enzyme known as short-chain acyl-CoA dehydrogenase, which is involved in the breakdown of short-chain fatty acids. (malacards.org)
Acyl-Coa Dehydrogenase, Short-Chain, Deficiency of, also known as scad deficiency , is related to acyl-coa dehydrogenase, very long-chain, deficiency of and myopathy , and has symptoms including seizures , lethargy and failure to thrive . (malacards.org)
An important gene associated with Acyl-Coa Dehydrogenase, Short-Chain, Deficiency of is ACADS (Acyl-CoA Dehydrogenase Short Chain), and among its related pathways/superpathways are Fatty acid degradation and Fatty acid metabolism . (malacards.org)
71 Acyl-CoA dehydrogenase short-chain deficiency: An inborn error of mitochondrial fatty acid beta-oxidation resulting in acute acidosis and muscle weakness in infants, and a form of lipid- storage myopathy in adults. (malacards.org)
Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. (abcam.com)
The Massachusetts Department of Public Health authorized the expansion of the mandated disorders to include medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. (thefreelibrary.com)
Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: iden tification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism. (thefreedictionary.com)
As a newborn-screening assay, MS/MS is not merely a method for detecting medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (7), nor is it simply an improved method for accurate neonatal detection of PKU with a false-positive rate 10-fold lower than the best method previously available (8). (thefreedictionary.com)
Molecular characterization of inherited medium-chain acyl-CoA dehydrogenase deficiency. (thefreedictionary.com)
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common disorder associated with fatty acid oxidation. (dovepress.com)
To understand how muscle FA oxidation affects systemic glucose, we studied mice with a skeletal muscle-specific deficiency of long-chain acyl-CoA synthetase (ACSL)1. (diabetesjournals.org)
Medium-chain acyl-coenzyme A dehydrogenase deficiency can be caused by mutations in the ACADM gene. (wikipedia.org)
More than 30 ACADM gene mutations that cause medium-chain acyl-coenzyme A dehydrogenase deficiency have been identified. (wikipedia.org)
With a shortage (deficiency) of functional ACADM enzyme, medium-chain fatty acids cannot be degraded and processed. (wikipedia.org)
Long Name: NADH dehydrogenase (NADH-CoQ reductase) deficiency. (umdf.org)
IJlst L, Wanders RJ, Ushikubo S, Kamijo T, Hashimoto T. Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein. (medscape.com)
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a severe fatty acid oxidation disorder. (medscape.com)
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid beta-oxidation. (medscape.com)
Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast. (medscape.com)
Clinical, biochemical, and morphologic investigations of a case of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. (medscape.com)
C rearrangement in patients with long chain 3-hydroxyacyl-CoA dehydrogenase deficiency from Ukraine. (medscape.com)
C of HADHA gene causing long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) in the population of adult Kashubians from North Poland. (medscape.com)
Clinical outcome, biochemical and therapeutic follow-up in 14 Austrian patients with Long-Chain 3-Hydroxy Acyl CoA Dehydrogenase Deficiency (LCHADD). (medscape.com)
Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood. (medscape.com)
Schiff M, Mohsen AW, Karunanidhi A, McCracken E, Yeasted R, Vockley J. Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. (medscape.com)
Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxy-acyl-CoA dehydrogenase deficiency. (medscape.com)
Pigmentary retinopathy in long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. (medscape.com)
Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia.The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. (avivasysbio.com)
Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. (avivasysbio.com)
Multiple acyl-CoA dehydrogenase deficiency (MADD) is a life-threatening, ultrarare inborn error of metabolism. (nature.com)
Mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency are long-chain fatty acid oxidation disorders with particularly high morbidity and mortality. (biomedcentral.com)
Both MTP deficiency and isolated LCHADD lead to an accumulation of toxic ß-oxidation intermediates causing acute symptoms as well as long-term complications. (biomedcentral.com)

Long-chain 3-hydroxy acyl-coenzyme A dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that comprise the trifunctional protein of the inner mitochondrial membrane. (medigest.uk)
This enzyme complex helps metabolize long-chain fatty acids, and the LCHAD activity is specific for compounds of C12-C16 chain length. (medigest.uk)
Serum amino acids were normal, but urinalysis demonstrated elevated levels of free dicarboxylic acids and derivatives, and long-chain 3-OH acyl CoA dehydrogenase (LCHAD) defect was considered. (aappublications.org)
See LCAD , LCHAD , MAD , MCAD , SCAD , SCHAD , VLCAD Treatment: High carbohydrate-low fat diet, administration of medium-chain triglyceride oil, and diet supplementation with carnitine and/or riboflavin. (umdf.org)
Cecatto C, Godoy KDS, da Silva JC, Amaral AU, Wajner M. Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle. (medscape.com)

Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress. (labome.org)
Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. (labome.org)
Our data suggest that carnitine supplementation in long-chain beta-oxidation defects may not be required, and blood carnitine concentrations do not reflect tissue carnitine homeostasis. (labome.org)
Dietary management consisted of skimmed milk supplemented with medium chain triglycerides and l -carnitine. (bmj.com)
At 1.5 months of age the patient was started on low fat milk formula (1% fat in dry powder) supplemented with carbohydrate, medium chain triglyceride (MCT) oil (1.5 g/kg/day), and l -carnitine 100 mg/kg/day. (bmj.com)
acyl-CoA molecules are first coupled to carnitine, catalysed by carnitine-palmitoyltransferase I (CPT 1), and the acylcarnitine complex then crosses the highly impermeable inner mitochondrial membrane. (hindawi.com)
In the mitochondrial matrix space, acylcarnitines are reconverted to acyl-CoA via carnitine-palmitoyltransferase 2 (CPT2) (Figure 1 ) and subsequently enter beta-oxidation [ 5 ]. (hindawi.com)
Carnitine palmitoyltransferase 1A (CPT1A) is a rate-limiting enzyme in the transport of long-chain fatty acids for β-oxidation. (bioportfolio.com)
A long-chain fatty acid ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids. (bioportfolio.com)
Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. (hmdb.ca)

The extent of the LCADH-flavin cofactor reduction observed with most substrates and the rate of the subsequent reoxidation with oxygen are markedly different from those found with human medium chain acyl-CoA dehydrogenase. (uni-konstanz.de)

Very-long-chain acyl-CoA dehydrogenase (EC 1.3.8.9, ACADVL (gene). (wikipedia.org)
The ACADVL gene provides instructions for making an enzyme called very long-chain acyl-CoA dehydrogenase (VLCAD). (medlineplus.gov)
Pan troglodytes acyl-CoA dehydrogenase very long chain (ACADVL), transcript variant X1, mRNA. (genscript.com)

ter Veld F, Primassin S, Hoffmann L, Mayatepek E, Spiekerkoetter U. Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice. (labome.org)
This is the first study demonstrating that acylcarnitines and acyl-CoA directly correlate and concomitantly increase after exercise in VLCAD-deficient muscle. (labome.org)
Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the initial rate-limiting step in mitochondrial fatty acid beta-oxidation. (nih.gov)
Babies with VLCAD cannot make certain fats into energy, especially during long periods without food (fasting). (newbornscreening.on.ca)
To prevent a metabolic crisis, babies with VLCAD must not go a long time without eating. (newbornscreening.on.ca)
VLCAD occurs when the body either does not make enough or makes non-working enzyme called very long-chain acyl-CoA dehydrogenase. (diseaseinfosearch.org)
In this case, the job of the VLCAD enzyme is to break down the very long-chain fatty acids, which are parts of the fat from our food. (diseaseinfosearch.org)
Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene. (semanticscholar.org)
Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of four straight-chain acyl-CoA dehydrogenase (ACD) enzymes, which are all nuclear encoded mitochondrial flavoproteins catalyzing the initial step in fatty acid beta-oxidation. (semanticscholar.org)
LC-MS/MS was used to measure MCAD- or VLCAD-catalyzed production of enoyl-CoA and hydroxyacyl-CoA, in human lymphocytes. (cdc.gov)
In this study, we analyzed VLCAD −/− mice under different metabolic conditions such as after moderate (1 h) and intensive long-term (1 h twice per day over 2 weeks) physical exercise and after 24 h of fasting. (springeropen.com)
To address this question, we measured the palmitoyl-CoA (C16-CoA) oxidation rate in the kidney of wild-type (WT) and VLCAD −/− mice in situations of normal and increased energy demand as well as the expression of other dehydrogenases with overlapping substrate specificity. (springeropen.com)

The other 2 activities of the protein are long-chain 3-ketoacyl CoA thiolase (LCKT) and 2-enoyl coenzyme A (CoA) hydratase (LCEH). (medigest.uk)

To discern whether two related nuclear genes are expressed similarly, the tissue distribution and developmental profile of the rat long- and medium-chain acyl-CoA dehydrogenase (LCAD and MCAD) mRNAs were compared. (elsevier.com)
MCAD works on long-chain fatty acids, typically between C4 and C12-acylCoA. (wikipedia.org)

Characterization of Chorioretinopathy Associated with Mitochondrial Trifunctional Protein Disorders : Long-Term Follow-up of 21 Cases. (ohsu.edu)
Sirtuin 3 (SIRT3) protein regulates long-chain acyl-CoA dehydrogenase by deacetylating conserved lysines near the active site. (duke.edu)
ACAD10 (Acyl-CoA Dehydrogenase Family Member 10) is a Protein Coding gene. (genecards.org)
In addition, acyl-CoA binding protein and some isoforms of FA binding protein can bind and carry acyl-CoAs. (diabetesjournals.org)
BPD, initially performed as an unclog procedure and then laparoscopically, is a extraordinarily personal property long-term pressure losing box office with some inherent risk of protein calorie and micronutrient malnutrition. (bonetumor.org)
However, long-term studies are needed in order to determine whether higher protein diets reduce risks of overweight and obesity. (medscape.com)
This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. (avivasysbio.com)

Recombinant adeno-associated virus-mediated gene delivery of long chain acyl coenzyme A dehydrogenase (LCAD) into LCAD-deficient mice. (umassmed.edu)
Long-chain acyl-CoA dehydrogenase (LCAD) is a key mitochondrial fatty acid oxidation enzyme. (duke.edu)

Long-chain acyl-CoA dehydrogenase (EC 1.3.8.8, palmitoyl-CoA dehydrogenase, palmitoyl-coenzyme A dehydrogenase, long-chain acyl-coenzyme A dehydrogenase, long-chain-acyl-CoA:(acceptor) 2,3-oxidoreductase, ACADL (gene). (wikipedia.org)
ACADL belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. (avivasysbio.com)

On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. III. (wikipedia.org)
This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. (nih.gov)
ACADM ( acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain ) is a gene that provides instructions for making an enzyme called acyl-coenzyme A dehydrogenase that is important for breaking down (degrading) a certain group of fats called medium-chain fatty acids . (wikipedia.org)
The acyl-coenzyme A dehydrogenase for medium-chain fatty acids (ACADM) enzyme is essential for converting these particular fatty acids to energy, especially during periods without food (fasting). (wikipedia.org)

hydroxyacyl-CoA dehydrogenase/3-ketoacyl-C. (broadinstitute.org)

MADD can be primary, caused by a genetic defect in the electron transfer flavoproteins (ETF) or in ETF dehydrogenase (ETFDH), or secondary, resulting from genetic defects of riboflavin transport (RFVT) or flavin adenine dinucleotide (FAD) synthesis (i.e. (nature.com)

Tandem mass spectrometry on dried blood spots revealed increased long chain acylcarnitines. (bmj.com)
The profile showed significantly increased myristoleylcarnitine (C14:1) at 5.6 μmol/l (upper cut off value 0.30 μmol/l) and increased palmitoylcarnitine (C16) at 20.2 μmol/l (cut off 9.30 μM) as well as several other long chain acylcarnitines. (bmj.com)
Moreover, we quantified the content of glycogen and long-chain acylcarnitines in the kidney. (springeropen.com)
We observed a significant depletion in renal glycogen with a concomitant reduction in long-chain acylcarnitines, suggesting a substrate switch for energy production and an optimal compensation of impaired fatty acid oxidation in the kidney. (springeropen.com)
Addition of medium-chain triglycerides (MCT) treatment (providing 10-20% of energy requirements) is reported to be beneficial with improvement in dicarboxylic aciduria and a normalization of the plasma level of long-chain acylcarnitines. (medscape.com)

Our results demonstrate that LCADH is as important as the other acyl-CoA dehydrogenases in fatty acid oxidation at physiological, mitochondrial pH with optimal substrates of chain length C10 C14. (uni-konstanz.de)
Very long-chain acyl-CoA dehydrogenase is essential for fatty acid oxidation, which is the multistep process that breaks down (metabolizes) fats and converts them to energy. (medlineplus.gov)
Gregersen N, Andresen BS, Corydon MJ, Corydon TJ, Olsen RK, Bolund L, Bross P. Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship. (medlineplus.gov)
Thus, the clinical features may arise from either toxicity due to long-chain acyl-CoA esters that cause cardiomyopathy and cardiac arrhythmias or from a block in long-chain fatty acid oxidation that leads to an inability to synthesize ketone bodies and/or adenosine triphosphate from long-chain fatty acids. (medigest.uk)
We measured the oxidation rate of palmitoyl-CoA (C16-CoA) as well as the expression of genes involved in lipogenesis and renal failure. (springeropen.com)
Acyl CoA dehydrogenase is the enzyme used to catalyze the first step of β-oxidation in Fatty acid metabolism . (wikidoc.org)
In this review, the physiology of energy metabolism in muscle is described, followed by the presentation of distinct disorders affecting skeletal and cardiac muscle: glycogen storage diseases types III, V, VII, fatty acid oxidation defects, and respiratory chain defects (i.e., mitochondriopathies). (hindawi.com)
Fatty acids are metabolized in the mitochondrial matrix to acetyl-CoA via beta-oxidation (Figures 1 and 2 ). (hindawi.com)
Adequate glucose supply, conversion of acetyl-CoA to malonyl-CoA by acetyl-CoA carboxylase, and the concomitant inhibition of CPT1 reduce long-chain fatty acid uptake and oxidation (Figure 2 ). (hindawi.com)
An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. (abcam.com)
CPT1a-Dependent Long-Chain Fatty Acid Oxidation Contributes to Maintaining Glucagon Secretion from Pancreatic Islets. (bioportfolio.com)
Previous immunometabolism research using the CPT1 inhibitor etomoxir suggests that long-chain fatty acid oxidation (LC-FAO) supports IL-4-driven alternative macrophage activation (M(IL-4)) and regulat. (bioportfolio.com)
β-Oxidation in ghrelin-producing cells is important for ghrelin acyl-modification. (bioportfolio.com)
The first step of the [beta]-oxidation cycle is catalyzed by acyl-CoA dehydrogenases , whereas the enzymes of MTP catalyze the last 3 steps of [beta]-oxidation. (thefreedictionary.com)
ACADM, a member of the acyl-CoA dehydrogenase (ACAD) family that comprises 9 known proteins, is involved in the oxidation of medium-chain fatty acids and amino acids (Kim Miura, 2004). (thefreedictionary.com)
Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders : a double blinded, randomized controlled trial. (ohsu.edu)
This gene encodes a member of the acyl-CoA dehydrogenase family of enzymes (ACADs), which participate in the beta-oxidation of fatty acids in mitochondria. (genecards.org)
Surprisingly, the content of muscle acyl-CoA at exhaustion was markedly elevated, indicating that acyl-CoAs synthesized by other ACSL isoforms were not available for β-oxidation. (diabetesjournals.org)
Before FAs are used for oxidation or for the synthesis of cholesterol esters, phospholipids, or triacylglycerol (TAG), they must be activated to form acyl-CoAs by one of a family of five long-chain acyl-CoA synthetases (ACSLs), each encoded by a separate gene ( 10 ). (diabetesjournals.org)

This gene provides instructions for making an enzyme called very long-chain acyl-CoA dehydrogenase, which is required to break down (metabolize) a group of fats called very long-chain fatty acids. (medlineplus.gov)
Knowledge of the profile of fatty acids, including the profile of long chain fatty acids, is an important parameter for many areas like human health, food sciences, and the study of gene expression in. (bioportfolio.com)

enoyl-CoA, hydratase/3-hydroxyacyl CoA de. (broadinstitute.org)

In my own specialist area of lipid metabolism, deficiencies of the fatty acid metabolizing acyl-CoA dehydrogenases are defined as a group of diseases that may be potentially treatable with a combination of dietary manipulation and prevention of fasting, because these are essentially diseases of fasting intolerance. (thefreedictionary.com)
Deficiencies have been identified in each of these FAO enzyme forms with the exception of long-chain acyl-CoA dehydrogenase (5). (thefreedictionary.com)
Complex I, the first step in this chain, is the most common site for mitochondrial abnormalities, representing as much as one third of the respiratory chain deficiencies. (umdf.org)

Other disorders, such as defects in pyruvate metabolism or in the respiratory chain, may present with metabolic acidosis and an elevated lactate during the neonatal period, but these may not always have an increased anion gap. (medscape.com)
Sodium phenylbutyrate decreases plasma branched-chain amino acids in patients with urea cycle disorders. (ohsu.edu)

Purification and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. (wikipedia.org)
Pig kidney LCADH was purified using an optimized method which also produces apparently pure short-chain-acyl-CoA dehydrogenase (SCADH) and medium-chain-acyl-CoA dehydrogenase (MCADH) in good yields. (uni-konstanz.de)

Within the spiral, enzymes with overlapping chain-length specificities catalyze each step. (dovepress.com)
Medium-chain acyl-CoAs generated by long-chain specific enzymes at the inner mitochondrial membrane undergo a series of enzymatic steps in the matrix to generate acetyl-CoA and a chain-shortened acyl-CoA. (dovepress.com)

Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. (nih.gov)
Both LCADH are inactivated by the substrate analogue 2-octynoyl-CoA, possibly via covalent modification of GIu261, the active-site residue involved in deprotonation of the substrate (α)C-H. (uni-konstanz.de)
Thus, an acyl-CoA should be able to enter any downstream pathway, unless the entry into one specific pathway versus another depend on the relative substrate affinities of the initiating acyltransferases. (diabetesjournals.org)

The most common amino acid substitution replaces lysine with glutamic acid at position 329 in the enzyme's chain of amino acids (also written as Lys329Glu or K329E). (wikipedia.org)

is an enzyme with systematic name long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase. (wikipedia.org)
This enzyme catalyses the following chemical reaction a long-chain acyl-CoA + electron-transfer flavoprotein ⇌ {\displaystyle \rightleftharpoons } a long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein This enzyme contains FAD as prosthetic group and participates in fatty acid metabolism and PPAR signaling pathway. (wikipedia.org)
A flavoprotein oxidoreductase that has specificity for long-chain fatty acids. (umassmed.edu)
Identification and diagnosis of diseases of this flavoprotein (ETF) dehydrogenase. (bokus.com)

Without sufficient amounts of this enzyme, very long-chain fatty acids are not metabolized properly. (medlineplus.gov)
The human enzyme has an approximately fivefold higher Km, compared with the pig kidney enzyme with substrates of chain length from C10, to C18, and a significantly different dependence of Vmax on the chain length. (uni-konstanz.de)

Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. (nih.gov)

[ 37 ] Use of flax/walnut oils (containing the least amount of nonessential fatty acids) when compared to canola oil may reduce the accumulation of disease specific acyl-CoA intermediates, preventing peripheral neuropathy. (medscape.com)

Goetzman ES, Wang Y, He M, Mohsen AW, Ninness BK, Vockley J. Expression and characterization of mutations in human very long-chain acyl-CoA dehydrogenase using a prokaryotic system. (medlineplus.gov)
In vitro characterization and in vivo expression of human very-long chain acyl-CoA dehydrogenase. (medlineplus.gov)

Selective Inhibition of Acyl-CoA Dehydrogenases by a Metabolite of Hypoglycin. (thefreedictionary.com)

Most patients also are provided with uncooked cornstarch and medium-chain triglyceride (MCT) oil supplementation to further decrease exposure to fasting. (medscape.com)

Inside the mitochondrion is a group of proteins that carry electrons along four chain reactions (Complexes I-IV), resulting in energy production. (umdf.org)

acyl-CoAs are amphipathic molecules that are both soluble in water and able to integrate into membrane monolayers via their hydrophobic acyl chains. (diabetesjournals.org)

Although these effects have primarily been shown in liver and heart, there is also evidence for a regulatory function of malonyl-CoA in skeletal muscle [ 4 ]. (hindawi.com)
Clinical symptoms mainly develop during periods of illness or fasting and affect organs preferring long-chain fat as primary source of energy, such as heart and skeletal muscle. (biomedcentral.com)

Only leucine and isoleucine can be oxidised in muscle after being converted to acetyl CoA. (hindawi.com)

Anatomy5

Organisms1

Diseases2

Chemicals and Drugs83

Analytical, Diagnostic and Therapeutic Techniques and Equipment5

Phenomena and Processes13

Technology, Industry, Agriculture1

Information Science3

Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death. (1/138)

The medium-/long-chain fatty acyl-CoA dehydrogenase (fadF) gene of Salmonella typhimurium is a phase 1 starvation-stress response (SSR) locus. (2/138)

Cloning and mapping of three pig acyl-CoA dehydrogenase genes. (3/138)

Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. (4/138)

Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids. (5/138)

Effect of endurance training on lipid metabolism in women: a potential role for PPARalpha in the metabolic response to training. (6/138)

Mitochondrial transcription factor A is increased but expression of ATP synthase beta subunit and medium-chain acyl-CoA dehydrogenase genes are decreased in hearts of copper-deficient rats. (7/138)

Production of fatty acid components of meadowfoam oil in somatic soybean embryos. (8/138)

3-Hydroxyacyl CoA Dehydrogenases

Acyl-CoA Dehydrogenases

Acyl-CoA Dehydrogenase

Acyl Coenzyme A

Diacylglycerol O-Acyltransferase

Sterol O-Acyltransferase

L-Lactate Dehydrogenase

Acyltransferases

Coenzyme A

Alcohol Dehydrogenase

Fatty Acids

Glyceraldehyde-3-Phosphate Dehydrogenases

Coenzyme A Ligases

Aldehyde Dehydrogenase

Glutamate Dehydrogenase

Glucosephosphate Dehydrogenase

Malate Dehydrogenase

Isocitrate Dehydrogenase

Alcohol Oxidoreductases

Molecular Sequence Data

Acyl-CoA Oxidase

Triazenes

Kinetics

Diazepam Binding Inhibitor

Palmitoyl Coenzyme A

Dihydrolipoamide Dehydrogenase

Succinate Dehydrogenase

Amino Acid Sequence

Carbohydrate Dehydrogenases

L-Iditol 2-Dehydrogenase

Oxidoreductases

Glycerolphosphate Dehydrogenase

Liver

Aldehyde Oxidoreductases

NAD

Acyl-CoA Dehydrogenase, Long-Chain

Oxidation-Reduction

Glucose 1-Dehydrogenase

Hydroxysteroid Dehydrogenases

Ketoglutarate Dehydrogenase Complex

Fatty Acids, Unsaturated

Palmitic Acids

Substrate Specificity

Sugar Alcohol Dehydrogenases

3-Hydroxysteroid Dehydrogenases

Glucose Dehydrogenases

Cholesterol Esters

Phosphogluconate Dehydrogenase

NADH Dehydrogenase

Oleic Acids

Esterification

Microsomes

IMP Dehydrogenase

Base Sequence

Lactate Dehydrogenases

Polymerase Chain Reaction

Formate Dehydrogenases

Lipid Metabolism

17-Hydroxysteroid Dehydrogenases

Triglycerides

Microbodies

Xanthine Dehydrogenase

Hydroxybutyrate Dehydrogenase

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

Acylation

Cloning, Molecular

Ketone Oxidoreductases

Carnitine O-Palmitoyltransferase

Pyruvate Dehydrogenase (Lipoamide)

NADP

Escherichia coli

Cholesterol

RNA, Messenger

11-beta-Hydroxysteroid Dehydrogenases

Acetyl Coenzyme A

Lipids

Adrenoleukodystrophy

Uridine Diphosphate Glucose Dehydrogenase

Dihydrouracil Dehydrogenase (NADP)

Butyryl-CoA Dehydrogenase