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 ... "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Homo ...

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

"Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction". The Journal of Biological ... "ACADM - Medium-chain specific acyl-CoA dehydrogenase, mitochondrial precursor - Homo sapiens (Human) - ACADM gene & protein". ... Wang SS, Fernhoff PM, Hannon WH, Khoury MJ (1999). "Medium chain acyl-CoA dehydrogenase deficiency human genome epidemiology ... "Molecular cloning of cDNAs encoding rat and human medium-chain acyl-CoA dehydrogenase and assignment of the gene to human ...

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

... 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

"Identification and characterization of new long chain acyl-CoA dehydrogenases". Molecular Genetics and Metabolism. 102 (4): 418 ... Acyl-CoA dehydrogenase family, member 10 is a protein that in humans is encoded by the ACAD10 gene. This gene encodes a member ... "Entrez Gene: Acyl-CoA dehydrogenase family, member 10". Bian L, Hanson RL, Muller YL, Ma L, Kobes S, Knowler WC, Bogardus C, ... of the acyl-CoA dehydrogenase family of enzymes (ACADs), which participate in the beta-oxidation of fatty acids in mitochondria ...

https://en.wikipedia.org/wiki/ACAD10

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

... 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

... 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

Acyl-CoA dehydrogenases are enzymes that catalyze formation of a double bond between C2 (α) and C3 (β) of the acyl-CoA ... N-acylethanolamine (NAE) Abedi E, Sahari MA (September 2014). "Long-chain polyunsaturated fatty acid sources and evaluation of ... Thorpe C, Kim JJ (June 1995). "Structure and mechanism of action of the acyl-CoA dehydrogenases". FASEB Journal. 9 (9): 718-25 ... Plant stearoyl-acyl-carrier-protein desaturase (EC 1.14.19.1), an enzyme that catalyzes the introduction of a double bond at ...

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

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

"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

... 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

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

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

... 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

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, 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) Portal: Biology (EC ...

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

... 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

... (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) Portal: Biology ( ... 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

... 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

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 ... "Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)". "VLCAD deficiency , Genetic and Rare Diseases Information Center ( ... Mutations in the ACADVL gene lead to inadequate levels of an enzyme called very long-chain acyl-coenzyme A (CoA) dehydrogenase ...

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

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

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

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

Fatty acids are made by fatty acid synthases that polymerize and then reduce acetyl-CoA units. The acyl chains in the fatty ... Lipids are the polymers of fatty acids[citation needed] that contain a long, non-polar hydrocarbon chain with a small polar ... Hundreds of separate types of dehydrogenases remove electrons from their substrates and reduce NAD+ into NADH. This reduced ... the acetyl group on acetyl-CoA is oxidized to water and carbon dioxide in the citric acid cycle and electron transport chain, ...

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

CoA) dehydrogenase, which is part of a protein complex known as mitochondrial trifunctional protein. Long-chain fatty acids ... Medium chain acyl dehydrogenase deficiency Reference, Genetics Home. "LCHAD deficiency". Genetics Home Reference. Retrieved ... "Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: Clinical Presentation and Follow-Up of 50 Patients". Pediatrics. 109 (1 ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency is a rare autosomal recessive fatty acid oxidation disorder that ...

https://en.wikipedia.org/wiki/Long-chain_3-hydroxyacyl-coenzyme_A_dehydrogenase_deficiency

"The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-oxoacyl-CoA ... which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons shorter. The encoded protein can also bind ... long-chain enoyl-CoA hydratase, and long-chain thiolase. This deficiency can be classified into 3 main clinical phenotypes: ... Trifunctional protein deficiency is characterized by decreased activity of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), ...

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

By disrupting an acyl-coenzyme A (CoA) thioesterase gene, Sabirova and colleagues were able to mutate the organism to hyper- ... a product that consists of a long chain of carbon atoms attached to hydrogen atoms. Whereas most organisms use sugars or amino ... This sequential pathway first produces alcohols, then alcohol and aldehyde dehydrogenases, and ultimately aldehydes and fatty ...

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

The catechol is then metabolized to acetyl CoA and succinyl CoA, used by organisms mainly in the citric acid cycle for energy ... Long-term exposure to excessive levels of benzene in the air causes leukemia, a potentially fatal cancer of the blood-forming ... On p. 100, Kekulé suggests that the carbon atoms of benzene could form a "chaîne fermée" (a closed chain, a loop). Aug. Kekulé ... The reaction involves the acylation of benzene (or many other aromatic rings) with an acyl chloride using a strong Lewis acid ...

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

Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain fatty acids. When this ... "Disorders of mitochondrial fatty acyl-CoA beta-oxidation" (PDF). Journal of Inherited Metabolic Disease. 22 (4): 442-487. doi: ... caused by long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. This leads to decreased metabolism of long chain fatty ... IJlst L, Oostheim W, Ruiter JP, Wanders RJ (1997). "Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: ...

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

SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... AKAP9 Long QT syndrome-3; 603830; SCN5A Long QT syndrome-4; 600919; ANK2 Long QT syndrome-7; 170390; KCNJ2 Long QT syndrome-9; ... TGFBR2 Long QT syndrome 12; 612955; SNT1 Long QT syndrome 13; 613485; KCNJ5 Long QT syndrome-1; 192500; KCNQ1 Long QT syndrome- ...

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

It has been found in certain Candida yeast, where it participates in omega oxidation of fatty acids to produce acyl-CoA for ... Yeast have low levels of fatty alcohol dehydrogenase.) The long-chain alcohol is then oxidized by long-chain fatty aldehyde ... Long-chain alcohol oxidase catalyzes the chemical reaction long-chain alcohol + O2 ⇌ {\displaystyle \rightleftharpoons } 2 long ... Long-chain fatty alcohol oxidases participate in omega-oxidation of long chain alkanes or fatty acids. The alkane is first ...

https://en.wikipedia.org/wiki/Long-chain-alcohol_oxidase

... acyl-CoA oxidase (see, e.g., ACOX1, MIM 609751); the 'D-bifunctional enzyme,' with enoyl-CoA hydratase and D-3-hydroxyacyl-CoA ... short-chain dehydrogenase/reductase and related enzymes) nomenclature initiative". Chemico-Biological Interactions. 178 (1-3): ... v t e (Articles with short description, Short description matches Wikidata, Genes on human chromosome 5, CS1: long volume value ... "Purification and properties of human D-3-hydroxyacyl-CoA dehydratase: medium-chain enoyl-CoA hydratase is D-3-hydroxyacyl-CoA ...

https://en.wikipedia.org/wiki/HSD17B4

Isobutyryl-CoA and Isovaleryl-CoA respectively. In bacteria, this enzyme participates in the synthesis of branched, long-chain ... STEP 3: Acyl group transfer to CoA. The proper arrow-pushing mechanism is shown in Figure 5. *NOTE: The reduced lipoyl arm now ... Other mitochondrial autoantigens include pyruvate dehydrogenase and branched-chain oxoglutarate dehydrogenase, which are ... The branched-chain α-ketoacid dehydrogenase complex (BCKDC or BCKDH complex) is a multi-subunit complex of enzymes that is ...

https://en.wikipedia.org/wiki/Branched-chain_alpha-keto_acid_dehydrogenase_complex

... 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 ... Jones JM, Gould SJ (Aug 2000). "Identification of PTE2, a human peroxisomal long-chain acyl-CoA thioesterase". Biochemical and ... 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/ACOT4

This system is distinct from the branched-chain fatty acid synthetase that utilizes short-chain acyl-CoA esters as primers. α- ... propionyl-CoA instead of acetyl-CoA is used as the primer for the biosynthesis of long-chain fatty acids with an odd number of ... the cycle as CO2 in the decarboxylation reactions catalyzed by isocitrate dehydrogenase and alpha-ketoglutarate dehydrogenase. ... Regulation Acetyl-CoA is formed into malonyl-CoA by acetyl-CoA carboxylase, at which point malonyl-CoA is destined to feed into ...

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

Acetate-CoA ligase EC 6.2.1.2: Medium-chain acyl-CoA ligase EC 6.2.1.3: Long-chain-fatty-acid-CoA ligase EC 6.2.1.4: Succinate- ... EC 1.1.1.10 Lactate dehydrogenase EC 1.1.1.27 Malate dehydrogenase EC 1.1.1.37 Isocitrate dehydrogenase EC 1.1.1.42 HMG-CoA ... Citrate-CoA ligase EC 6.2.1.19: Long-chain-fatty-acid-luciferin-component ligase EC 6.2.1.20: Long-chain-fatty-acid-(acyl- ... Medium-chain-fatty-acid-(acyl-carrier-protein) ligase EC 6.2.1.48: Carnitine-CoA ligase EC 6.2.1.49: Long-chain fatty acid ...

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

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

"Misfolding of short-chain acyl-CoA dehydrogenase leads to mitochondrial fission and oxidative stress". Molecular Genetics and ... Pain processing is no longer seen as a repetitive relay of signals from body to brain, but as a complex system that can be up- ... Long-term potentiation: Scientists debate whether astrocytes integrate learning and memory in the hippocampus. Recently it has ... In their results they were able to see that astrocytes had a direct role in Long-term potentiation with the mixed culture ( ...

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

The conversion of malonic acid to malonyl-CoA by acyl-CoA synthetase represents the first step in the mitochondrial fatty acid ... "Mammalian ACSF3 Protein Is a Malonyl-CoA Synthetase That Supplies the Chain Extender Units for Mitochondrial Fatty Acid ... such as pyruvate dehydrogenase complex (PDHC) and α-ketoglutarate dehydrogenase complex (α-KGDHC), among others. This ... which may contribute to neurological symptoms in the long term. Methylmalonic acid is formed from the essential amino acids ...

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

Increased malonyl-CoA level pushes the equilibrium over to increase production of fatty acids through biosynthesis. Long chain ... Pyruvate dehydrogenase (PDH), converts pyruvate into acetyl-CoA. Acetyl-CoA carboxylase (ACC), converts acetyl-CoA produced by ... Fatty acid esterification takes place in the endoplasmic reticulum of cells by metabolic pathways in which acyl groups in fatty ... Affecting ACC affects the rate of acetyl-CoA conversion to malonyl-CoA. ...

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

... 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 ... These enzymes have also been referred to in the literature as acyl-CoA hydrolases, acyl-CoA thioester hydrolases, and palmitoyl ... coenzyme A These enzymes use the same substrates as long-chain acyl-CoA synthetases, but have a unique purpose in that they ...

https://en.wikipedia.org/wiki/ACOT1

Using esterification of long-chain fatty acids, excess propionyl-CoA can be sequestered and stored in the lipid, ... PCC not only catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA, but also acts on several different acyl-CoAs. ... For example, inhibition of pyruvate dehydrogenase by an accumulation of propionyl-CoA in Rhodobacter sphaeroides can prove ... Therefore, odd-number chains of fatty acids are oxidized to yield both propionyl-CoA as well as acetyl-CoA. Propionyl-CoA is ...

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

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/

Very long-chain acyl CoA dehydrogenase deficiency is an inherited disorder of mitochondrial oxidation of long-chain fatty acids ... Very long chain acyl-CoA dehydrogenase deficiency (VLCADD) Very long chain acyl CoA dehydrogenase deficiency (VLCADD) is an ... Very long chain acyl-CoA dehydrogenase deficiency (VLCADD) ... inherited disorder of the mitochondrial oxidation of long chain ... Mutations in this gene cause dysfunction of mitochondrial beta-oxidation of long chain fatty acids. ...

https://www.tellmegen.com/en/results/monogenic-diseases/very-long-chain-acyl-coa-dehydrogenase-deficiency-vlcadd

Very long chain acyl-CoA dehydrogenase deficiency. Disclaimer: The disease-specific care plans and shared datasets described ... herein have been prepared solely to establish uniform data collection for measurement of long-term follow-up outcome measures. ...

https://www.mountainstatesgenetics.org/projects/former-projects/metabolic-consortium/long-chain-acyl-coa-dehydrogenase-deficiency/

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

3-methylcrotonyl-CoA carboxylase deficiency (3MCC)*. Homocystinuria (HCU)*. Long-chain hydroxyl acyl-CoA dehydrogenase ... 3-methylcrotonyl-CoA carboxylase deficiency (3MCC)*. Homocystinuria (HCU)*. Long-chain hydroxyl acyl-CoA dehydrogenase ... Short chain acyl-CoA dehydrogenase deficiency (SCAD)*. Isobutyl CoA dehydrogenase deficiency Maple syrup urine disease (MSUD)* ... Short chain acyl-CoA dehydrogenase deficiency (SCAD)*. Isobutyl CoA dehydrogenase deficiency Maple syrup urine disease (MSUD)* ...

https://www.medscape.com/viewarticle/493247_4

Potential brain injury occurring during these episodes can lead to an increased risk of long term neurological damage. Sudden ... Medium chain acyl-CoA dehydrogenase deficiency. Disease definition Medium chain acyl-CoA dehydrogenase (MCAD) deficiency (MCADD ... Differential diagnosis includes other disorders of mitochondrial fatty acid oxidation including multiple acyl-CoA dehydrogenase ... Medium chain triglycerides should be avoided but no other special dietary restrictions are required. Guidelines are available ...

https://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=3570&Disease_Disease_Search_diseaseGroup=42&Disease_Disease_Search_diseaseType=ORPHA&Disease

Short Chain Acyl-CoA Dehydrogenase Deficiency. SCAD. Trifunctional Protein Deficiency. TFP. Very Long Chain Acyl-CoA ... Medium/Short Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency. M/SCHAD. ... Medium Chain Acyl-CoA Dehydrogenase Deficiency. MCAD. ... Isobutyryl-CoA dehydrogenase deficiency. IBG. Long Chain ...

https://portal.ct.gov/Newborn-Screening-Program/Pages/CT-NBS-Panel

Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD). fatty acid oxidation. yes. yes. yes. ... Long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency (LCHAD). fatty acid oxidation. yes. yes. yes. ... Medium-chain acyl-CoA dehydrogenase deficiency (MCAD). fatty acid oxidation. yes. yes. yes. ... Short-chain acyl-CoA dehydrogenase deficiency. fatty acid oxidation. no. yes. yes. ...

https://www.chop.edu/pages/newborn-metabolic-screenings-pa-nj-and-de

... is essential for the assembly of mitochondrial respiratory chain complex I. Disease causing biallelic variants in ACAD9 have ... ACAD9 is most homologous (47% amino acid identity, 65% amino acid similarity) to very long-chain acyl-CoA dehydrogenase (VLCAD ... Mitochondrial acyl-CoA dehydrogenase family member 9 (ACAD9) is essential for the assembly of mitochondrial respiratory chain ... by a double-blind randomized crossover study of bezafibrate in five individuals with acyl-CoA dehydrogenase very long chain ( ...

https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0784-8

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https://rgd.mcw.edu/rgdweb/ontology/view.html?acc_id=DOID:3651

Very Long Chain Acyl-CoA Dehydrogenase Deficiency. *Costa CG, Struys EA, Bootsma A, ten Brink HJ, Dorland L, Tavares de Almeida ... isovalerylcarnitine is found to be associated with celiac disease and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD ... Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem ... Belongs to the class of organic compounds known as acyl carnitines. These are organic compounds containing a fatty acid with ...

https://hmdb.ca/metabolites/HMDB0000688

Ever heard of medium-chain acyl-CoA dehydrogenase deficiency? As youll have guessed, its a rare metabolic disorder - ... But the Herceptin debate is certainly not over - the benefit is small, the cardiac dangers great, the long-term effects unknown ...

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2007) that ACAD9 and very long-chain acyl-CoA dehydrogenase (ACADVL; 609575) are unable to compensate for each other in ... Acyl-CoA dehydrogenase 9 (ACAD9) is an assembly factor for mitochondrial respiratory chain Complex I (CI). The tissue ... Acyl-CoA dehydrogenase 9 deficiency General Information (adopted from Orphanet):. Synonyms, Signs: ACAD9 deficiency. ... All 3 patients had biochemical findings suggestive of an unknown long-chain fat metabolism defect. ...

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Ryder, 3, has VLCAD, which stands for very long-chain acyl-CoA dehydrogenase deficiency. It "is a condition in which the body ... called very long-chain fatty acids) into energy, particularly during periods without food (fasting)," according to the Genetic ... Has It Really Been That Long? A Look Back at How the Teen Mom Stars Have Transformed Over 10 Years ...

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... in the gene that encodes for mitochondrial long-chain hydroxy acyl-CoA dehydrogenase (LCHAD). The mutation in LCHAD results in ... and long-chain hydroxy acyl-CoA dehydrogenase (LCHAD) deficient children [9,10,11,12]. Further, long-chain 3-hydroxy ... The α-subunit contains long chain enoyl-CoA hydratase activity in its amino-terminal domain, while long chain hydroxy acyl-CoA ... Enoyl-CoA hydratase in the α-subunit catalyzes the conversion of enoyl-CoA to 3-hydroxy acyl-CoA. Next, 3-hydroxy acyl-CoA is ...

https://www.mdpi.com/1422-0067/19/1/322

It plays an essential role in the transfer of long-chain fatty acids into the mitochondria for beta-oxidation. ... such as LCHAD or very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, which may cause secondary carnitine deficiency. ... Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder. Pediatrics. ... In some disorders (eg, MCAD, LCHAD, short-chain acyl-CoA dehydrogenase [SCAD] deficiency) specific patterns can be seen. ...

https://emedicine.medscape.com/article/942233-workup

Very Long Chain Acyl CoA Dehydrogenase Deficiency (VLCAD). *Screen Positive Follow-Up ... Treatment for Tyrosinemia Type 1 begins as early as possible and is life long. ... Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD). *Maple Syrup Urine Disease ...

https://www.newbornscreening.on.ca/en/disease/tyrosinemia-type-1

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Ryder is diagnosed with VLCAD ("very long-chain acyl-CoA dehydrogenase deficiency") which means "she cant process fatty foods ... "As long as were communicating and Ryders happy, thats all we really care about," Floyd told Us in January. "Thats very ...

https://www.usmagazine.com/celebrity-news/news/how-teen-moms-cheyenne-cory-rely-on-each-other-as-coparents/

The long-chain specific acyl-CoA dehydrogenase (ACADL) is specially identified in the tetracycline group that catalyzes the ... D. M. Kurtz, P. Rinaldo, W. J. Rhead et al., "Targeted disruption of mouse long-chain acyl-CoA dehydrogenase gene reveals ... the overload lipid in hepatic cytoplasm activates the long-chain-fatty-acid-CoA ligase 1 and promotes the production of ... However, lower dose for longer time of exposure did not induce liver steatosis [21]. The toxicity of INH is mediated through ...

https://www.hindawi.com/journals/bmri/2019/1283824/

... acyl-coenzyme A dehydrogenase short chain (ACADS), long-chain acyl-CoA dehydrogenase (LCAD), and very long-chain acyl-CoA ... We found significant decrease in C-4 to C-12 straight chain of acyl-coA dehydrogenases (ACADM) and C-2 to C-3 short chain of ... and very long-chain acyl-CoA dehydrogenase (VLCAD) at transcript level as compared to control (Figure 1A). Furthermore, we also ... we also found significant decrease in other biomarkers of fatty acid oxidation like long-chain acyl-CoA dehydrogenase (LCAD), ...

https://www.frontiersin.org/articles/10.3389/fcvm.2021.574708/full

... medium chain, deficiency of that can make a difference in your life or the life of someone you love with alternative treatments ... People with MCADD cannot refrain from eating for very long. Fasting starts after the body has used all the food that the person ... Acyl-CoA dehydrogenase, medium chain, deficiency of in Texas. Acyl-CoA dehydrogenase, medium chain, deficiency of in Utah. Acyl ... Acyl-CoA dehydrogenase, medium chain, deficiency of by state. Acyl-CoA dehydrogenase, medium chain, deficiency of in Alabama. ...

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... acyl-CoA dehydrogenase which converts a fatty acyl-CoA into a fatty alcohol and CoASH. ... longer chain alcohols (C12-C20), though the mouse enzyme also used decanol as a substrate. Long chain alcohols are also ... effective part of the enzyme complex accumulate long chain fatty alcohols. The mammalian alcohol dehydrogenase system is a ... cycle producing acetyl-CoA products (and a propionyl‑CoA from odd chain-length molecules) and ATP. These enzymes are found in ...

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Search for abbreviations and long forms in lifescience, results along with the related PubMed / MEDLINE information and co- ... acyl-CoA dehydrogenase long chain. (13 times) ≫. Endocrinology. (2 times). HCC (2 times). NEFA (2 times). OS (2 times). ≫ ... Long Form No.. Long Form. Research Area. Co-occurring Abbreviation. PubMed/MEDLINE Info. (Year, Title). ...

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Very Long Chain Acyl-Coa Dehydrogenase Deficiency. Tachycardia, Arrhythmia, Dilated cardiomyopathy, Ventricular tachycardia, ...

https://www.mousephenotype.org/data/genes/MGI:104755

Transcription of acyl-CoA dehydrogenase genes (both long-chain ACADL and medium-chain ACADM) was suppressed at 48 h, while ... long-chain fatty acid, CoA ligase 1), responsible for the conversion of free long-chain fatty acids into fatty acyl-CoA esters ... Very long-chain acyl-CoA synthetases. Human "bubblegum" represents a new family of proteins capable of activating very long- ... acyl-CoA dehydrogenases and CPT1 (responsible for transport and oxidation of long-chain fatty acids in mitochondria) were ...

https://journals.biologists.com/bio/article/7/8/bio034629/2139/Role-of-murine-macrophage-in-temporal-regulation

Long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency - LCHAD. 53. Medium-chain acyl-CoA dehydrogenase deficiency - MCAD. 53 ... Medium-chain ketoacyl-CoA thiolase deficiency - MCKAT. 19. 17. 17. Medium/short-chain L-3-hydroxyacl-CoA dehydrogenase ... Short-chain acyl-CoA dehydrogenase deficiency - SCAD. 13. 32. 8. Hemoglobin Disorders. ...

https://www.newsteps.org/data-resources/reports/screened-conditions-report

Clinician and Patient Perspectives in the Management of Long-Chain Fatty Acid Oxidation Disorders 0.75 CME Credits ... Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD) * Sections Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency ... encoded search term (Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD)) and Medium-Chain Acyl-CoA Dehydrogenase ( ... Medium-chain acyl-CoA dehydrogenase deficiency in children with non- ketotic hypoglycemia and low carnitine levels. Pediatr Res ...

https://emedicine.staging.medscape.com/article/946755-workup

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Etomoxir (inhibitor of long chain FA) also limits FA availability to mitochondria, thus, less FAO (β-oxidation) further limits Acetyl-CoA production (substrate for TCA cycle). (frontiersin.org)
RELMα partially increases glycolytic oxidation and increases pyruvate dehydrogenase kinase (PDK-1, 4) gene expression which also inhibits conversion of pyruvate to acetyl-CoA for TCA cycle. (frontiersin.org)
Very long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats (PubMed:7668252, PubMed:9461620, PubMed:18227065, PubMed:9839948, PubMed:9599005). (cansar.ai)
18 - 21 However, MCTs rapidly cross the mitochondrial membrane and enter metabolic pathways to be beta-oxidized, producing a larger amount of acetyl-CoA than carbohydrates (CHOs). (jomes.org)
18 - 21 The liver mitochondria convert additional acetyl-CoA into ketone bodies beta-hydroxybutyrate (BOHB), acetoacetate (AcAc), and acetate (Ac) or oxalacetate. (jomes.org)
To produce energy G-6-PO 4 (derived from monosaccharides from dietary CHO or produced from glycogen degradation by glycogenolysis) is converted by a series of enzyme reactions in the glycolytic pathway to form pyruvate or lactic acid, then to acetyl-CoA, which is also produced from fatty acid oxidation and degradation of the carbon skeleton of glucogenic amino acids ( Table 17.1 ). (oncohemakey.com)
Acetyl-CoA enters the Krebs cycle, also known as the citric acid or tricarboxylic (TCA) cycle, within the mitochondria. (oncohemakey.com)
Within the Krebs cycle acetyl-CoA, combined with oxaloacetate, undergoes cycles involving eight enzymes, in which reducing equivalents are produced which then enter the electron transfer chain for the production of energy as adenosine triphosphate (ATP). (oncohemakey.com)
Dietary fats, and lipids produced endogenously from acetyl-CoA, are initially hydrolysed by lipases into glycerol and free fatty acids. (oncohemakey.com)
Glycerol is then oxidised to acetyl-CoA via pyruvate. (oncohemakey.com)
Fatty acids enter the mitochondria via the carnitine transport cycle (medium chain fatty acids enter independently of carnitine) into the β-oxidation spiral in which fatty acids, via a series of enzymes, produce acetyl-CoA and electron carriers. (oncohemakey.com)
Acetyl-CoA can enter the Krebs cycle or form ketone bodies in the liver. (oncohemakey.com)
Acetyl-CoA in excess of requirements for energy production via the Krebs cycle is converted via lipogenesis to stored lipids in adipocytes. (oncohemakey.com)
Beta-oxidation of long chain fatty acids produces two carbon units, acetyl-CoA and the reducing equivalents NADH and FADH2. (selfdecode.com)
Acetyl-CoA enters the Krebs Cycle and is also used to make ATP via the electron transport chain and substrate level phosphorylation. (selfdecode.com)
When the supply of acetyl-CoA (coming from the beta-oxidation of fatty acids) exceeds the capacity of the Krebs Cycle to metabolize acetyl-CoA, the excess acetyl-CoA molecules are converted to ketone bodies (acetoacetate and beta-hydroxybutyrate) by HMG-CoA synthase in the liver. (selfdecode.com)

Among the different mitochondrial acyl-CoA dehydrogenases, very long-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 12 to 24 carbons long primary chains (PubMed:21237683, PubMed:9839948). (cansar.ai)

Medium chain acyl-CoA dehydrogenase (MCAD) deficiency (MCADD) is an inborn error of mitochondrial fatty acid oxidation characterized by a rapidly progressive metabolic crisis, often presenting as hypoketotic hypoglycemia, lethargy, vomiting, seizures and coma, which can be fatal in the absence of emergency medical intervention. (orpha.net)
Urinary organic acid profile usually is normal in these patients when they are well, except in cases of medium-chain 3-hydroxyacyl-CoA dehydrogenase (MCAD) deficiency. (medscape.com)
In some disorders (eg, MCAD, LCHAD, short-chain acyl-CoA dehydrogenase [SCAD] deficiency) specific patterns can be seen. (medscape.com)
Measurement of serum electrolyte levels may reveal depressed bicarbonate and an anion gap in medium-chain acyl-coenzyme A (CoA) dehydrogenase (MCAD) deficiency. (medscape.com)
Medium-chain fatty acids accumulating in MCAD deficiency elicit lipid and protein oxidative damage and decrease non-enzymatic antioxidant defenses in rat brain. (medscape.com)
The enzyme MCAD (medium-chain acyl-CoA dehydrogenase) is responsible for the dehydrogenation step of fatty acids with chain lengths between 6 and 12 carbons as they undergo beta-oxidation in the mitochondria. (selfdecode.com)
MCAD works on long-chain fatty acids, typically between C4 and C12-acylCoA. (huabio.com)

Other fatty acid oxidation disorders, such as long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, can present with liver involvement. (medscape.com)
Elevated lactate can be observed in respiratory chain defects or in LCHAD deficiency. (medscape.com)
Modest amounts of long-chain 3-hydroxy fatty acids consistently are found in the plasma of patients with LCHAD deficiency, even if these patients are asymptomatic. (medscape.com)
These are carnitine palmitoyltransferase I (CPT I) deficiency, carnitine-acyl-carnitine translocase (CACT) deficiency, carnitine palmitoyltransferase II (CPT II) deficiency, very long-chain acyl-coenzyme A (CoA) dehydrogenase (VLCAD) deficiency, long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, and trifunctional protein (TFP) deficiency. (rarediseaseadvisor.com)
In the present study, a team of researchers led by Al-Walid Mohsen, PhD, from the University of Pittsburgh tested medium branched-chain fatty acids as potential anaplerotic treatments in fibroblasts obtained from patients with CPT II deficiency, VLCAD deficiency, LCHAD deficiency, and TFP deficiency. (rarediseaseadvisor.com)

Recently, this was weakened by a double-blind randomized crossover study of bezafibrate in five individuals with acyl-CoA dehydrogenase very long chain (ACADVL) deficiencies in whom no improvement could be detected [ 11 ]. (biomedcentral.com)

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)
Very-long-chain acyl-coenzyme A dehydrogenase deficiency--a new cause of myoglobinuric acute renal failure. (managementguidelines.net)
Reversal of severe hypertrophic cardiomyopathy and excellent neuropsychologic outcome in very-long-chain acyl-coenzyme A dehydrogenase deficiency. (managementguidelines.net)
Fatty acyl coenzyme A dehydrogenase (ambiguous), Acyl coenzyme A dehydrogenase (ambiguous), Acyl dehydrogenase (ambiguous), Fatty-acyl-CoA dehydrogenase (ambiguous), Acyl CoA dehydrogenase (ambiguous), General acyl CoA dehydrogenase (ambiguous), Medium-chain acyl-coenzyme A dehydrogenase, Acyl-CoA:(acceptor) 2,3-oxidoreductase (ambiguous), ACADM (gene name). (umassmed.edu)
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. (huabio.com)
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). (huabio.com)

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)
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)
Without sufficient amounts of this enzyme, very long-chain fatty acids are not broken down properly. (medlineplus.gov)
Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (EC 1.3.99.10) resulting in the accumulation of derivatives of isovaleryl-CoA. (hmdb.ca)
Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD) is a rare hereditary disease that is the result of the lack of an enzyme required to convert fat to energy. (naturalcurefor.com)
Risk stratification by residual enzyme activity after newborn screening for medium-chain acyl-CoA dehyrogenase deficiency: data from a cohort study. (medscape.com)
[1] 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. (rareimmunology.com)
Each type is caused by a mutation encoding a different enzyme involved in the β-oxidation of long-chain fatty acids. (rarediseaseadvisor.com)
In HMG-CoA lyase deficiency, lack of the enzyme 3-hydroxy-3-methylglutaryl-CoA lyase (usually expressed at high levels in the liver) impairs the formation of ketone bodies from fat and the breakdown of leucine. (medicalhomeportal.org)

Differential diagnosis includes other disorders of mitochondrial fatty acid oxidation including multiple acyl-CoA dehydrogenase deficiency (MADD) (see this term). (orpha.net)
Acylcarnitine profile and free fatty acid levels: Tandem mass spectrometry analyses of acylcarnitine profile and free fatty acids may be used to detect metabolic defects that cause secondary carnitine deficiency (eg, fatty acid oxidation disorders, organic acidemias) because acyl-CoA intermediates proximal to the block in fatty acid or amino acid oxidative pathway may be transesterified to carnitine. (medscape.com)
Intravenous Sources of Medium Chain Triglycerides for Critically Ill Patients with Fatty Acid Oxidation Disorders. (managementguidelines.net)
Substrate oxidation and cardiac performance during exercise in disorders of long chain fatty acid oxidation. (managementguidelines.net)
Medium branched-chain fatty acids could be a viable anaplerotic treatment option for long-chain fatty acid oxidation disorders (LCFAOD), according to a new study published in the Journal of Inherited Metabolic Disease . (rarediseaseadvisor.com)
The researchers concluded, "The results provide the impetus to further evaluate and consider branched-chain fatty acids as viable anaplerotic therapy for fatty acid oxidation disorders and other diseases. (rarediseaseadvisor.com)
De Biase I, Gherasim C, La'ulu SL, Asamoah A, Longo N, Yuzyuk T . Laboratory Evaluation of Homocysteine Remethylation Disorders and Classic Homocystinuria: Long-Term Follow-Up Using a Cohort of 123 Patients. (aruplab.com)

On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A. III. (wikipedia.org)
The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (PubMed:7668252, PubMed:9461620, PubMed:18227065, PubMed:9839948). (cansar.ai)

Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations . (rareimmunology.com)
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. (rareimmunology.com)

Purification and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. (wikipedia.org)
2] Another study showed that in two different cell lines, aspirin increased mitochondrial long-chain fatty acid oxidation, did not change oxidation of medium chain fatty acids, and inhibited peroxisomal fatty acid oxidation, which suggest that aspirin impairs long-chain fatty acid transport into mitochondria. (medscape.com)

Population spectrum of ACADM genotypes correlated to biochemical phenotypes in newborn screening for medium-chain acyl-CoA dehydrogenase deficiency. (medscape.com)

Mutations in ACADS have been associated with Short Chain Acyl-CoA Dehydrogenase Deficiency. (prospecbio.com)

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)
ACAD9 is most homologous (47% amino acid identity, 65% amino acid similarity) to very long-chain acyl-CoA dehydrogenase (VLCAD). (biomedcentral.com)
16602101 ). Moreover, isovalerylcarnitine is found to be associated with celiac disease and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD), which are also inborn errors of metabolism. (hmdb.ca)
Ryder, 3, has VLCAD , which stands for very long-chain acyl-CoA dehydrogenase deficiency. (intouchweekly.com)
Ryder is diagnosed with VLCAD ("very long-chain acyl-CoA dehydrogenase deficiency") which means "she can't process fatty foods into energy," Wharton explained to Us in December 2017. (usmagazine.com)
Effects of IV glucose and oral medium-chain triglyceride in patients with VLCAD deficiency. (managementguidelines.net)

C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects. (medscape.com)

Mutations in the HMGCL gene cause HMG-CoA lyase deficiency. (medicalhomeportal.org)
See HMG-CoA Lyase Deficiency - Information for Parents (STAR-G) . (medicalhomeportal.org)

Very long-chain fatty acids or partially metabolized fatty acids may also build up in tissues and damage the heart, liver, and muscles. (medlineplus.gov)
Very long chain acyl CoA dehydrogenase deficiency (VLCADD) is an inherited disorder of the mitochondrial oxidation of long chain fatty acids with variable phenotypes which include: cardiomyopathy, hypocetotic hypoglycemia, liver disease, exercise intolerance and rhabdomyolysis. (tellmegen.com)
It has been demonstrated that octanoic (OA) and decanoic (DA) acids compromise the glycolytic pathway and citric acid cycle functioning, increase oxygen consumption in the liver and inhibit some activities of the respiratory chain complexes and creatine kinase in rat brain (A15454, A15455). (selfdecode.com)

Medium-chain acyl-CoA dehydrogenase deficiency in children with non- ketotic hypoglycemia and low carnitine levels. (medscape.com)
12 , 16 , 17 Unlike long-chain triglycerides (LCTs), MCTs can easily pass through the mitochondrial membrane without carnitine palmitoyl transferase or a shuttle system. (jomes.org)

Mutations in this gene cause dysfunction of mitochondrial beta-oxidation of long chain fatty acids. (tellmegen.com)

Calvaresi EC, Genzen JR . Evaluating Percentage-Based Reporting of Glucose-6-Phosphate Dehydrogenase (G6PD) Enzymatic Activity. (aruplab.com)

Disclaimer: The disease-specific care plans and shared datasets described herein have been prepared solely to establish uniform data collection for measurement of long-term follow-up outcome measures. (mountainstatesgenetics.org)
Mitochondrial acyl-CoA dehydrogenase family member 9 (ACAD9) is essential for the assembly of mitochondrial respiratory chain complex I. Disease causing biallelic variants in ACAD9 have been reported in individuals presenting with lactic acidosis and cardiomyopathy. (biomedcentral.com)
Medium-chain triglyceride (MCT) oil has been reported as a therapeutic aid for Alzheimer disease, epilepsy, and malabsorption. (jomes.org)

Acyl-CoA dehydrogenase 9 (ACAD9) is an assembly factor for mitochondrial respiratory chain Complex I (CI). (helmholtz-muenchen.de)

NADH production utilized by oxidative phosphorylation (OXPHOS) pathway for ATP production through electron transport chain. (frontiersin.org)
NADH and FADH2 enter the electron transport chain and are used to make ATP. (selfdecode.com)

ACADs belong to a family of flavoenzymes involved in the ß-oxidation of acyl-CoA and amino acid catabolism. (biomedcentral.com)
ACADS is a tetrameric mitochondrial flavoprotein, which is part of the acyl-CoA dehydrogenase family. (prospecbio.com)
ACADS Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 409 amino acids (25-412 a.a.) and having a molecular mass of 44 kDa. (prospecbio.com)

Belongs to the class of organic compounds known as acyl carnitines. (hmdb.ca)

Newborn screening for medium chain acyl-CoA dehydrogenase deficiency in England: prevalence, predictive value and test validity based on 1.5 million screened babies. (medscape.com)

Defects in long chain fatty acid oxidation presenting as severe cardiomyopathy and cardiogenic shock in infancy. (managementguidelines.net)
One study demonstrated that salicylates decrease beta-oxidation of the long-chain fatty acid palmitate by cultured fibroblasts from children who recovered from Reye syndrome as compared with control subjects. (medscape.com)

Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism. (medscape.com)

People with MCADD cannot refrain from eating for very long. (naturalcurefor.com)

Belongs to the acyl-CoA dehydrogenase family. (huabio.com)

Riboflavin is the precursor of flavin adenine dinucleotide (FAD) and flavin mononucleotide (FMN), which are cofactors for complex I and numerous dehydrogenases involved in FAO. (biomedcentral.com)

Medium chain triglycerides should be avoided but no other special dietary restrictions are required. (orpha.net)
A suggested enteral feeding products is casein hydrolysate, supplemented with medium-chain acyl-coa dehydrogenase deficiency using tandem mass spectrometry for newborn screening. (aaan.org)
Medium-chain acyl-CoA dehydrogenase deficiency in Saudi Arabia: incidence, genotype, and preventive implications. (medscape.com)
Newborn screening for medium-chain acyl-CoA dehydrogenase deficiency: a global perspective. (medscape.com)
Medium-chain acyl-coA dehydrogenase deficiency: evaluation of genotype-phenotype correlation in patients detected by newborn screening. (medscape.com)
Abnormal screening in a healthy infant of a mother with undiagnosed medium-chain acyl-coA dehydrogenase deficiency. (medscape.com)
acyl-CoA synthetase medium chain fa. (gsea-msigdb.org)
The use of medium-chain triglyceride (MCT) oil has increased due to its potential for therapeutic and ergogenic properties. (jomes.org)
Medium-chain fatty acids are also produced when larger fatty acids are degraded. (huabio.com)
Anderson DR, Viau K, Botto LD, Pasquali M, Longo N . Clinical and biochemical outcomes of patients with medium-chain acyl-CoA dehydrogenase deficiency. (aruplab.com)

Electron carriers (FADH 2 and NAD) enter the electron transfer chain to produce ATP. (oncohemakey.com)

Isabella Linz, 8, of Meadville, who was diagnosed with very long-chain acyl-COA dehydrogenase deficiency, a life-threatening condition that prevents the body from converting certain fats to energy, saw her wish come true as she was given a trip for her and her family to Hawaii to swim with the dolphins. (edinboro.edu)
Aldehyde dehydrogenase family [Interproscan]. (ntu.edu.sg)

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