Acyl coa dehydrogenase. 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.

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).

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.

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.

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 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 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.

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

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)

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)

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)

The rate dynamics in chemical or physical systems.

Oxidoreductases that are specific for ALDEHYDES.

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.-.

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

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

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.

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

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.

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

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 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.

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

Alcohol oxidoreductases with substrate specificity for LACTIC ACID.

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.

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.

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.

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

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).

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.

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)

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 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).

Oxidoreductases that are specific for KETONES.

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)

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.

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

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

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.

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.

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

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

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

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.

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.

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

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

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

Catalyzes reversibly the oxidation of hydroxyl groups of prostaglandins.

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 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.

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.

The addition of an organic acid radical into a molecule.

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.

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).

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 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)

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).

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

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.

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 cloning of cDNA encoding mitochondrial very-long-chain acyl-CoA dehydrogenase from bovine heart. (1/257)

AIM: To clone the cDNA encoding an isoenzyme of mitochondrial very-long-chain acyl-CoA dehydrogenase (VLCAD) from bovine heart lambda gt11 and lambda gt10 cDNA libraries. METHODS: The clone was isolated with immunoscreening technique and validated by (1) the microsequences of the N-terminus and three internal proteolytic fragments from the purified enzyme; (2) identification of the acyl-CoA dehydrogenase (AD) signature sequence; and (3) high homology of the deduced peptide sequences, as expected, with those of rat liver mitochondrial VLCAD. RESULTS: The cDNA (2203 bp) corresponds to a approximately 2.4-kb mRNA band from the same tissue source revealed by a Northern blotting. The deduced peptide sequence of 655 amino acids (70,537 Da) is composed of a 40-amino acid mitochondrial leader peptide moiety (4,346 Da) and a 615-amino acid peptide as a mature protein (66,191 Da). A comparison of the peptide sequences in the AD family shows the major diversity in their signal sequences, suggesting a structural basis for their different mitochondrial locations. The catalytic sites are all highly conserved among VLCAD. Ser-251 analogous to and Cys-215 diversified to other family members. A pseudo-consensus sequence of leucine zipper was found in the C-terminal region from Leu-568 to Leu-589, implying a mechanism whereby the dimer of this protein is formed by zipping these leucine residues from the alpha-helixes of 2 monomers. CONCLUSION: The isolated cDNA clone encodes an isoenzyme of mitochondrial VLCAD in bovine heart. (+info)

The medium-/long-chain fatty acyl-CoA dehydrogenase (fadF) gene of Salmonella typhimurium is a phase 1 starvation-stress response (SSR) locus. (2/257)

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)

Oxidation of medium-chain acyl-CoA esters by extracts of Aspergillus niger: enzymology and characterization of intermediates by HPLC. (3/257)

The activities of beta-oxidation enzymes were measured in extracts of glucose- and triolein-grown cells of Aspergillus niger. Growth on triolein stimulated increased enzyme activity, especially for acyl-CoA dehydrogenase. No acyl-CoA oxidase activity was detected. HPLC analysis after incubation of triolein-grown cell extracts with decanoyl-CoA showed that beta-oxidation was limited to one cycle. Octanoyl-CoA accumulated as the decanoyl-CoA was oxidized. Beta-oxidation enzymes in isolated mitochondrial fractions were also studied. The results are discussed in the context of methyl ketone production by fungi. (+info)

Outcome of medium chain acyl-CoA dehydrogenase deficiency after diagnosis. (4/257)

BACKGROUND: Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common inborn error of fatty acid metabolism. Undiagnosed, it has a mortality rate of 20-25%. Neonatal screening for the disorder is now possible but it is not known whether this would alter the prognosis. OBJECTIVE: To investigate the outcome of MCAD deficiency after the diagnosis has been established. METHOD: All patients with a proved diagnosis of MCAD deficiency attending one centre in a four year period were reviewed. RESULTS: Forty one patients were identified. Follow up was for a median of 6.7 years (range, 9 months to 14 years). Nearly half of the patients were admitted to hospital with symptoms characteristic of MCAD deficiency before the correct diagnosis was made. After diagnosis, two patients were admitted to hospital with severe encephalopathy but there were no additional deaths or appreciable morbidity. There was a high incidence (about one fifth) of previous sibling deaths among the cohort. CONCLUSIONS: Undiagnosed, MCAD deficiency results in considerable mortality and morbidity. However, current management improves outcome, supporting the view that the disorder should be included in newborn screening programmes. (+info)

A novel acyl-CoA oxidase that can oxidize short-chain acyl-CoA in plant peroxisomes. (5/257)

Short-chain acyl-CoA oxidases are beta-oxidation enzymes that are active on short-chain acyl-CoAs and that appear to be present in higher plant peroxisomes and absent in mammalian peroxisomes. Therefore, plant peroxisomes are capable of performing complete beta-oxidation of acyl-CoA chains, whereas mammalian peroxisomes can perform beta-oxidation of only those acyl-CoA chains that are larger than octanoyl-CoA (C8). In this report, we have shown that a novel acyl-CoA oxidase can oxidize short-chain acyl-CoA in plant peroxisomes. A peroxisomal short-chain acyl-CoA oxidase from Arabidopsis was purified following the expression of the Arabidopsis cDNA in a baculovirus expression system. The purified enzyme was active on butyryl-CoA (C4), hexanoyl-CoA (C6), and octanoyl-CoA (C8). Cell fractionation and immunocytochemical analysis revealed that the short-chain acyl-CoA oxidase is localized in peroxisomes. The expression pattern of the short-chain acyl-CoA oxidase was similar to that of peroxisomal 3-ketoacyl-CoA thiolase, a marker enzyme of fatty acid beta-oxidation, during post-germinative growth. Although the molecular structure and amino acid sequence of the enzyme are similar to those of mammalian mitochondrial acyl-CoA dehydrogenase, the purified enzyme has no activity as acyl-CoA dehydrogenase. These results indicate that the short-chain acyl-CoA oxidases function in fatty acid beta-oxidation in plant peroxisomes, and that by the cooperative action of long- and short-chain acyl-CoA oxidases, plant peroxisomes are capable of performing the complete beta-oxidation of acyl-CoA. (+info)

Cloning and mapping of three pig acyl-CoA dehydrogenase genes. (6/257)

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)

Peroxisome proliferator-activated receptor alpha mediates the adaptive response to fasting. (7/257)

Prolonged deprivation of food induces dramatic changes in mammalian metabolism, including the release of large amounts of fatty acids from the adipose tissue, followed by their oxidation in the liver. The nuclear receptor known as peroxisome proliferator-activated receptor alpha (PPARalpha) was found to play a role in regulating mitochondrial and peroxisomal fatty acid oxidation, suggesting that PPARalpha may be involved in the transcriptional response to fasting. To investigate this possibility, PPARalpha-null mice were subjected to a high fat diet or to fasting, and their responses were compared with those of wild-type mice. PPARalpha-null mice chronically fed a high fat diet showed a massive accumulation of lipid in their livers. A similar phenotype was noted in PPARalpha-null mice fasted for 24 hours, who also displayed severe hypoglycemia, hypoketonemia, hypothermia, and elevated plasma free fatty acid levels, indicating a dramatic inhibition of fatty acid uptake and oxidation. It is shown that to accommodate the increased requirement for hepatic fatty acid oxidation, PPARalpha mRNA is induced during fasting in wild-type mice. The data indicate that PPARalpha plays a pivotal role in the management of energy stores during fasting. By modulating gene expression, PPARalpha stimulates hepatic fatty acid oxidation to supply substrates that can be metabolized by other tissues. (+info)

Evaluating newborn screening programmes based on dried blood spots: future challenges. (8/257)

A UK national programme to screen all newborn infants for phenylketonuria was introduced in 1969, followed in 1981 by a similar programme for congenital hypothyroidism. Decisions to start these national programmes were informed by evidence from observational studies rather than randomised controlled trials. Subsequently, outcome for affected children has been assessed through national disease registers, from which inferences about the effectiveness of screening have been made. Both programmes are based on a single blood specimen, collected from each infant at the end of the first week of life, and stored as dried spots on a filter paper or 'Guthrie' card. This infrastructure has made it relatively easy for routine screening for other conditions to be introduced at a district or regional level, resulting in inconsistent policies and inequitable access to effective screening services. This variation in screening practices reflects uncertainty and the lack of a national framework to guide the introduction and evaluation of new screening initiatives, rather than geographical variations in disease prevalence or severity. More recently, developments in tandem mass spectrometry have made it technically possible to screen for several inborn errors of metabolism in a single analytical step. However, for each of these conditions, evidence is required that the benefits of screening outweigh the harms. How should that evidence be obtained? Ideally policy decisions about new screening initiatives should be informed by evidence from randomised controlled trials but for most of the conditions for which newborn screening is proposed, large trials would be needed. Prioritising which conditions should be formally evaluated, and developing a framework to support their evaluation, poses an important challenge to the public health, clinical and scientific community. In this chapter, issues underlying the evaluation of newborn screening programmes will be discussed in relation to medium chain acyl CoA dehydrogenase deficiency, a recessively inherited disorder of fatty acid oxidation. (+info)

"Multiple acyl-CoA dehydrogenase deficiency". Orphanet. INSERM and the European Commission. Retrieved 30 August 2018. "Glutaric ... "Highly efficient ketone body treatment in multiple acyl-CoA dehydrogenase deficiency-related leukodystrophy". Pediatr Res. 77 ( ... L-3-hydroxybutyrate treatment of multiple acyl-CoA dehydrogenase deficiency (MADD)". The Lancet. 361 (9367): 1433-5. doi: ... while the ETFDH gene encodes the enzyme electron transfer flavoprotein dehydrogenase. When one of these enzymes is defective or ...

https://en.wikipedia.org/wiki/Glutaric_acidemia_type_2

"Acyl-CoA dehydrogenases, electron transfer flavoprotein and electron transfer flavoprotein dehydrogenase". Biochemical Society ... A crystal structure of the complex of one of its interactors, medium-chain acyl-CoA dehydrogenase (MCAD; gene name ACADM) has ... Crane FL, Beinert H (September 1954). "A Link Between Fatty Acyl CoA Dehydrogenase and Cytochrome C: A New Flavin Enzyme". ... Defects in either of the ETF subunits or ETFDH cause multiple acyl CoA dehydrogenase deficiency (OMIM # 231680), earlier called ...

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

"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 ... Acyl CoA dehydrogenase GRCh38: Ensembl release 89: ENSG00000072778 - Ensembl, May 2017 GRCm38: Ensembl release 89: ... "Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency". American Journal of ...

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

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 ... "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 ... GeneReviews/NCBI/NIH/UW entry on Short-Chain Acyl-CoA Dehydrogenase Deficiency Human ACADS genome location and ACADS gene ...

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

Roth, Karl S. (2013-12-19). "Medium-Chain Acyl-CoA Dehydrogenase Deficiency". Medscape. Beermann, C.; Jelinek, J.; Reinecker, T ... The cytosolic acetyl-CoA is carboxylated by acetyl CoA carboxylase into malonyl-CoA, the first committed step in the synthesis ... Pyruvate is then decarboxylated to form acetyl-CoA in the mitochondrion. However, this acetyl CoA needs to be transported into ... To obtain cytosolic acetyl-CoA, citrate (produced by the condensation of acetyl-CoA with oxaloacetate) is removed from the ...

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

"Very long-chain acyl-CoA dehydrogenase deficiency". Genetics Home Reference, National Institutes of Health. Retrieved 5 January ...

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

"Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... CS1 maint: discouraged parameter (link) "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase ( ... HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, ALPHA SUBUNIT; HADHA". omim.org. Retrieved 5 ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ...

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

Acyl-CoA dehydrogenases are enzymes that catalyze formation of a double bond between C2 (α) and C3 (β) of the acyl-CoA ... 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 ... Family 1 includes Stearoyl-CoA desaturase-1 (SCD) (EC 1.14.19.1). Family 2 is composed of: Bacterial fatty acid desaturases. ...

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

Wang SS, Fernhoff PM, Hannon WH, Khoury MJ (1999). "Medium chain acyl-CoA dehydrogenase deficiency human genome epidemiology ... "Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction". The Journal of Biological ... "Molecular cloning of cDNAs encoding rat and human medium-chain acyl-CoA dehydrogenase and assignment of the gene to human ... Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship". Human Mutation. 18 ...

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

Short/branched chain acyl-CoA dehydrogenase (ACADSB) is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze ... an enzyme in the acyl CoA dehydrogenase family. It can cause short/branched-chain acyl-CoA dehydrogenase deficiency. The human ... "Entrez Gene: acyl-CoA dehydrogenase, short/branched chain". Andresen BS, Christensen E, Corydon TJ, Bross P, Pilgaard B, ... The cDNA is significantly similar to the cDNA of other members of the acyl-CoA dehydrogenase family; its structure is closest ...

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

"Cloning of nitroalkane oxidase from Fusarium oxysporum identifies a new member of the acyl-CoA dehydrogenase superfamily". Proc ... a carbanion-forming flavoprotein homologous to acyl-CoA dehydrogenase". Arch. Biochem. Biophys. 433 (1): 157-65. doi:10.1016/j. ...

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

Ikeda Y, Dabrowski C, Tanaka K (25 January 1983). "Separation and properties of five distinct acyl-CoA dehydrogenases from rat ... Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase". J. Biol. Chem. 258 (2): 1066-76. doi:10.1016/S0021- ... as it accepts electrons from multiple acetyl-CoA dehydrogenases. In plants, ETF-Q oxidoreductase is also important in the ... NADH dehydrogenase succinate dehydrogenase Coenzyme Q - cytochrome c reductase cytochrome c oxidase Metabolism portal. ...

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

... 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 is a protein that in humans is encoded by the ACADL gene. ACADL is a gene that encodes LCAD ... "Entrez Gene: Acyl-CoA dehydrogenase, long chain". Kurtz DM, Tolwani RJ, Wood PA (May 1998). "Structural characterization of the ...

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

"Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain". Biochemical and ... Acyl-CoA dehydrogenase family member 9, mitochondrial is an enzyme that in humans is encoded by the ACAD9 gene. Mitochondrial ... "Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids". The Journal ... "Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I". Cell Metabolism. 12 (3): 283- ...

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

"Evidence for involvement of medium chain acyl-CoA dehydrogenase in the metabolism of phenylbutyrate". Molecular Genetics and ... In the human body it is first converted to phenylbutyryl-CoA and then metabolized by mitochondrial beta-oxidation, mainly in ...

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

Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase. „J. Biol. Chem.". 258 (2), s. 1066-76, 1983. PMID: ... Ikeda Y, Dabrowski C, Tanaka K. Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria ... Alternative NAD(P)H dehydrogenases of plant mitochondria. „Annual review of plant biology". 55, s. 23-39, 2004. DOI: 10.1146/ ... Dervartanian DV, Veeger C.. Studies on succinate dehydrogenase. I. Spectral properties of the purified enzyme and formation of ...

https://pl.wikipedia.org/wiki/Fosforylacja_oksydacyjna

"The deuterium isotope effect upon the reaction of fatty acyl-CoA dehydrogenase and butyryl-CoA". J. Biol. Chem. 255 (19): 9093- ...

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

The inhibition of one in particular, butyryl CoA dehydrogenase (a short-chain acyl-CoA dehydrogenase), causes β-oxidation to ... MCPA also inhibits the dehydrogenation of a number of Acyl-CoA dehydrogenases. ... MCPA forms non-metabolizable esters with coenzyme A (CoA) and carnitine, causing a decrease in their bioavailability and ... it also limits Acyl and carnitine cofactors, which are instrumental in the oxidation of large fatty acids. Hypoglycin A ...

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

Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase". J. Biol. Chem. 258 (2): 1066-76. PMID 6401712.. ... "Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. ... Rasmusson AG, Soole KL, Elthon TE (2004). "Alternative NAD(P)H dehydrogenases of plant mitochondria". Annual review of plant ... Dervartanian DV, Veeger C. (1964). "Studies on succinate dehydrogenase. I. Spectral properties of the purified enzyme and ...

https://id.wikipedia.org/wiki/Fosforilasi_oksidatif

Also, it inhibits acyl-CoA dehydrogenases, so that only unsaturated fatty acids can be fully oxidized. Fatty acids accumulate ...

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

Medium chain acyl-CoA dehydrogenase deficiency (MCADD), which had been implicated in several cases of sudden infant death ... Prior to its inclusion in newborn screening, short-chain acyl-CoA dehydrogenase deficiency (SCADD) was thought to be life- ... "Homozygosity for a severe novel medium-chain acyl-CoA dehydrogenase (MCAD) mutation IVS3-1G>C that leads to introduction of a ... "Prenatal diagnosis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in a family with a previous fatal case of sudden ...

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

Fatty acyl CoA dehydrogenase requires FAD in fatty acid oxidation. *FAD is required to convert retinol (vitamin A) to retinoic ... and branched-chain amino acids requires FAD in the shared E3 portion of their respective dehydrogenase complexes ... acid via cytosolic retinal dehydrogenase. *Synthesis of an active form of folate (5-methyltetrahydrofolate) from 5,10- ...

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

Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase". J. Biol. Chem. 258 (2): 1066-76. PMID 6401712. ... "Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. ... as it accepts electrons from multiple acetyl-CoA dehydrogenases.[31][32] In plants, ETF-Q oxidoreductase is also important in ... Competitive inhibitors of succinate dehydrogenase (complex II).[91]. Antimycin A Piscicide Complex III Binds to the Qi site of ...

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

... is broken down into shorter-chain fatty acids in the human liver by the long-chain acyl CoA dehydrogenase enzyme. ... Erucic acid is produced by elongation of oleic acid via oleoyl-coenzyme A and malonyl-CoA. ...

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

"The deuterium isotope effect upon the reaction of fatty acyl-CoA dehydrogenase and butyryl-CoA". J. Biol. Chem. 255 (19): 9093- ...

https://id.wikipedia.org/wiki/Hydrogen

caiA RNAs are found upstream of genes whose protein products function as acyl CoA dehydrogenases, although in one case the ... annotated substrate is butyryl-CoA. caiA RNAs might regulate these genes (i.e. function as cis-regulatory elements), but it is ...

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

... 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 ... This is catalyzed by acyl CoA dehydrogenase to produce trans-delta 2-enoyl CoA. It uses FAD as an electron acceptor and it is ...

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

... and acyl CoA dehydrogenase. FADH and FADH2 are reduced forms of FAD. FADH2 is produced as a prosthetic group in succinate ... Flavin mononucleotide is a prosthetic group found in, among other proteins, NADH dehydrogenase, E.coli nitroreductase and old ... dehydrogenase, an enzyme involved in the citric acid cycle. In oxidative phosphorylation, two molecules of FADH2 typically ...

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

Dihydroorotate dehydrogenase. *Coproporphyrinogen III oxidase. *Protoporphyrinogen oxidase. *Bilirubin oxidase. *Acyl-CoA ...

https://en.wikipedia.org/wiki/2-alkenal_reductase

ACSF3: encoding enzyme Acyl-CoA synthetase family member 3. *ACSM2B: encoding enzyme Acyl-coenzyme A synthetase ACSM2B, ... PDPR: encoding protein Pyruvate dehydrogenase phosphatase regulatory subunit. *PKDTS: Polycystic kidney disease, infantile ... ACSM3: encoding enzyme Acyl-coenzyme A synthetase ACSM3, mitochondrial 2. *ADHD1: Attention deficit-hyperactivity disorder, ...

https://en.wikipedia.org/wiki/Chromosome_16

transferase activity, transferring acyl groups. • 3-hydroxyacyl-CoA dehydrogenase activity. • RNA binding. • acetyl-CoA C- ... HADHB, ECHB, MSTP029, MTPB, TP-BETA, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional ... transferring acyl groups other than amino-acyl groups. • enoyl-CoA hydratase activity. • long-chain-3-hydroxyacyl-CoA ... The thiol is inserted between C-2 and C-3, which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons ...

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

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

https://ko.wikipedia.org/wiki/효소

Pyruvate is oxidized to acetyl-CoA and CO2 by the pyruvate dehydrogenase complex (PDC). The PDC contains multiple copies of ... When oxygen is present, acetyl-CoA is produced from the pyruvate molecules created from glycolysis. Once acetyl-CoA is formed, ... Out of the cytoplasm it goes into the Krebs cycle with the acetyl CoA. It then mixes with CO2 and makes 2 ATP, NADH, and FADH. ... The citric acid cycle is an 8-step process involving 18 different enzymes and co-enzymes.[6] During the cycle, acetyl-CoA (2 ...

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

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

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

Dor warrt denn Acyl-CoA for an Carnitin andockt un dör de Binnenmembran dörslüüst un dor denn in Acetyl-CoA umwannelt. In den ... En sunnerlichen Enzymkomplex (Pyruvat-Dehydrogenase) hölpt denn dor to, dat Pyruvat up to spleten un in Acetyl-CoA um to ... Anners kann Acetyl-CoA ok tostanne brocht weern dör den Afbo vun Fettsüer (β-Oxidation). Bi Deerter passeert dat ok in de ... Citratcyklus warrt ut dat Acetyl-CoA de gröttste Deel vun de Reduktschoonsäquivalente (NADH+H+, FADH2) herstellt, de denn ...

https://nds.wikipedia.org/wiki/Mitochondrien

... 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 ... "Succinyl-CoA:3-ketoacid CoA transferase deficiency". Genetics Home Reference. National Institute of Health. Retrieved 4 ...

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

... namely acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and thiolase. The cycle produces a new ... Fatty acids are first converted to acyl-CoA. Acyl-CoA is then degraded in a four-step cycle of dehydrogenation, hydration, ... The cytosolic acetyl-CoA can also condense with acetoacetyl-CoA to form 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) which is the ... Acetyl-CoA can be carboxylated in the cytosol by acetyl-CoA carboxylase, giving rise to malonyl-CoA, a substrate required for ...

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

to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... transferase activity, transferring acyl groups, acyl groups converted into alkyl on transfer. • citrate (Si)-synthase activity ... The enzyme is inhibited by high ratios of ATP:ADP, acetyl-CoA:CoA, and NADH:NAD, as high concentrations of ATP, acetyl-CoA, and ... This induces the enzyme to change its conformation, and creates a binding site for the acetyl-CoA. Only when this citroyl-CoA ...

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

"The deuterium isotope effect upon the reaction of fatty acyl-CoA dehydrogenase and butyryl-CoA". J. Biol. Chem. 255 (19): 9093- ...

https://en.wikipedia.org/wiki/E949

... is produced which is a building block for new fatty acids and can inhibit the transfer of the fatty acyl group from acyl CoA to ... Acetyl-CoA carboxylase (ACC) is a biotin-dependent enzyme that catalyzes the irreversible carboxylation of acetyl-CoA to ... The carboxyl group is transferred from biotin to acetyl CoA to form malonyl CoA in the second reaction, which is catalyzed by ... Malonyl-CoA decarboxylase. References[edit]. *^ a b c Tong L (August 2005). "Acetyl-coenzyme A carboxylase: crucial metabolic ...

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

Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase". 《J. Biol. Chem.》 258 (2): 1066-76. PMID 6401712. 29 ... "Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. ... 포유류에서 ETF:Q 산화환원효소 대사 경로는 여러 아실-CoA 탈수소효소로부터 전자를 받아들일 수 있기 때문에 지방산의 β 산화와 아미노산과 콜린의 이화작용에서 중요하다.[30][31] 식물에서 ETF:Q 산화환원효소는 ... 예를 들어, 식물에는 미토콘드리아 기질 쪽이 아닌 막 사이 공간 쪽에서 NADH를 산화시키는 외부 NAD(P)H 탈수소효소(external NAD(P)H dehydrogenase)가 있으며, 전자를 유비퀴논 풀에 전달한다.[41 ...

https://ko.m.wikipedia.org/wiki/산화적_인산화

Acyl-CoA → Lysophosphatidic acid + CoA. Glycerol-1-phosphatase removes the phosphate group of glycerol 3-phosphate to generate ... Glycerol-3-phosphate dehydrogenases are located both in the cytosol and the intermembrane face of mitochondrial inner membrane ... and another acyl group is then added on the sn-2 position making phosphatidic acids. ... with glycerol-3-phosphate dehydrogenase. DHAP and thus glycerol 3-phosphate is also possible to be synthesized from amino acids ...

https://en.wikipedia.org/wiki/Glycerol_3-phosphate

3-hydroxyacyl-CoA dehydrogenase. *3-hydroxybutyryl-CoA dehydrogenase. *Alcohol dehydrogenase. *Aldo-keto reductase *1A1 ... 3R)-3-hydroxyacyl-[acyl-carrier-protein] + NADP+ ⇌. {\displaystyle \rightleftharpoons }. 3-oxoacyl-[acyl-carrier-protein] + ... Other names in common use include beta-ketoacyl-[acyl-carrier protein](ACP) reductase, beta-ketoacyl acyl carrier protein (ACP ... In enzymology, a 3-oxoacyl-[acyl-carrier-protein] reductase (EC 1.1.1.100) is an enzyme that catalyzes the chemical reaction ...

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

3-Hydroxybutyryl-CoA dehydrogenase. *Branched-chain amino acid aminotransferase. *Branched-chain alpha-keto acid dehydrogenase ... Glutamine is formed if an ammonium ion attacks the acyl-phosphate intermediate, while glutamate is remade if water attacks the ... Methylglutaconyl-CoA hydratase. (See Template:Leucine metabolism in humans - this diagram does not include the pathway for β- ... Ammonium binds strongly to GS only if the acyl-phosphate intermediate is present. Ammonium, rather than ammonia, binds to GS ...

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

Dozens of ATP equivalents are generated by the beta-oxidation of a single long acyl chain.[20] The acetyl-CoA produced by beta- ... In the mitochondrion, pyruvate is oxidized by the pyruvate dehydrogenase complex to the acetyl group, which is fully oxidized ... In the presence of air and various cofactors and enzymes, fatty acids are degraded to acetyl-CoA. The pathway is called beta- ... Another malate dehydrogenase-catalyzed reaction occurs in the opposite direction, producing oxaloacetate and NADH from the ...

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

Acetyl-coA inhibits pyruvate dehydrogenase, while succinyl-CoA inhibits alpha-ketoglutarate dehydrogenase and citrate synthase ... Acyl-CoA is oxidized to trans-Enoyl-CoA while FAD is reduced to FADH2, which is similar to the oxidation of succinate to ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... Succinyl-CoA + GDP + Pi Succinate + CoA-SH + GTP Succinyl-CoA synthetase substrate-level. phosphorylation or ADP→ATP instead of ...

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

The reaction is catalysed by the enzymes pyruvate decarboxylase and alcohol dehydrogenase.[13] ... Acyl-CoA Fatty. acids Glyco-. sphingolipids Sphingolipids Waxes Polyunsaturated. fatty acids Neurotransmitters. & thyroid ...

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

In higher eukaryotes, δ-aminolevulinic acid, the key precursor to porphyrins, is biosynthesized from glycine and succinyl-CoA ... Glyoxylate is then oxidized by hepatic lactate dehydrogenase to oxalate in an NAD+-dependent reaction.[26] ... The acyl radical is glycyl. Contents. *1 History and etymology. *2 Production ...

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

Elovson J, Vagelos PR (1968). „Acyl carrier protein. X. Acyl carrier protein synthetase". J. Biol. Chem. 243 (13): 3603-11. ... Koenzim A (CoA, CoASH, HSCoA) je koenzim, poznat po svojoj ulozi u sintezi i oksidaciji masnih kiselina, i oksidaciji piruvata ... phosphopantetheinyl transferase activates 10-formyltetrahydrofolate dehydrogenase". J. Biol. Chem. 285 (3): 1627-33. PMC ... acetil-CoA) kao supstrat.[5] On se sastoji od cistamina, pantotenata, i adenozin-trifosfata. ...

https://sr.wikipedia.org/wiki/Koenzim_A

ATP-dependent carboxylation of acetyl-CoA to malonyl-CoA. Lipids are important in making up the cell membrane. ... For example, alcohol dehydrogenase which catalyses the transfer of a hydride ion from ethanol to NAD+ interacts with the ... The resulting hydroxide anion nucleophilically attacks the acyl-enzyme complex to form a second tetrahedral oxyanion ... Cyclohexanedione targets the Acetyl-CoA carboxylase which is involved in the first step of fat synthesis: ...

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

"Medium-Chain Acyl-CoA Dehydrogenase Deficiency". Medscape.. *^ Beermann, C.; Jelinek, J.; Reinecker, T.; Hauenschild, A.; Boehm ... The cytosolic acetyl-CoA is carboxylated by acetyl CoA carboxylase into malonyl-CoA, the first committed step in the synthesis ... To obtain cytosolic acetyl-CoA, citrate (produced by the condensation of acetyl-CoA with oxaloacetate) is removed from the ... Pyruvate is then decarboxylated to form acetyl-CoA in the mitochondrion. However, this acetyl CoA needs to be transported into ...

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

Dihydroorotate dehydrogenase. *Coproporphyrinogen III oxidase. *Protoporphyrinogen oxidase. *Bilirubin oxidase. *Acyl-CoA ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... to acetyl-CoA. *Pyruvate dehydrogenase complex (E1, E2, E3). *(regulated by Pyruvate dehydrogenase kinase and Pyruvate ... Succinate dehydrogenase cytochrome b560 subunit, mitochondrial. Pfam PF01127 4. SdhD. DHSD_HUMAN. Succinate dehydrogenase [ ...

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

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

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

Although these enzymes catalyze similar reactions and share a similar acyl-CoA dehydrogenase fold, the FMN-dependent enzyme is ...

https://en.wikipedia.org/wiki/4-Hydroxyphenylacetate_3-monooxygenase

Acyl CoA Beta oxidation Thorpe, C.; Kim, J. J. (June 1995). "Structure and Mechanism of Action of the Acyl-CoA Dehydrogenases ... "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 ... An additional class of acyl-CoA dehydrogenase was discovered that catalyzes α,β-unsaturation reactions with steroid-CoA ...

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

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, ... the two substrates of this enzyme are acyl-CoA and NADP+, whereas its 3 products are 2,3-dehydroacyl-CoA, NADPH, and H+. This ...

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

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

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

Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is a condition that prevents the body from converting certain fats into ... medlineplus.gov/genetics/condition/short-chain-acyl-coa-dehydrogenase-deficiency/ Short-chain acyl-CoA dehydrogenase deficiency ... Follow-up of patients with short-chain acyl-CoA dehydrogenase and isobutyryl-CoA dehydrogenase deficiencies identified through ... Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is a condition that prevents the body from converting certain fats into ...

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

Comment on: "Multiple acyl‑CoA dehydrogenase deficiency in elderly carriers". *Yılmaz Yıldız. ORCID: orcid.org/0000-0001-9076- ... Yıldız, Y., Tokatlı, A. Comment on: "Multiple acyl‑CoA dehydrogenase deficiency in elderly carriers". J Neurol (2020). https:// ... Grunert SC (2014) Clinical and genetical heterogeneity of late-onset multiple acyl-coenzyme A dehydrogenase deficiency. ... Multiple acyl-COA dehydrogenase deficiency in elderly carriers. J Neurol. https://doi.org/10.1007/s00415-020-09729-z ...

https://link.springer.com/article/10.1007%2Fs00415-020-09752-0

The PDB archive contains information about experimentally-determined structures of proteins, nucleic acids, and complex assemblies. As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards. The RCSB PDB also provides a variety of tools and resources. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists.

https://www.rcsb.org/pdb/protein/B4EGC8

VLCAD; Very long chain acyl-CoA dehydrogenase. COG1960. Location:80 → 451. CaiA; Acyl-CoA dehydrogenase related to the ... VLCAD; Very long chain acyl-CoA dehydrogenase. COG1960. Location:125 → 496. CaiA; Acyl-CoA dehydrogenase related to the ... VLCAD; Very long chain acyl-CoA dehydrogenase. COG1960. Location:26 → 397. CaiA; Acyl-CoA dehydrogenase related to the ... VLCAD; Very long chain acyl-CoA dehydrogenase. COG1960. Location:102 → 473. CaiA; Acyl-CoA dehydrogenase related to the ...

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

Catalyzes the dehydrogenation of acyl-CoA ester side chains of (25S)-3-oxo-cholest-4-en-26-oyl-CoA (3-OCS-CoA) to yield (24E)-3 ... 3-OCO-CoA) as well as 3-oxo-4-pregnene-20-carboxyl-CoA (3-OPC-CoA) (PubMed:26161441). It dehydrogenates only (25S)-OCS-CoA ... 25S-3-oxo-cholest-4-en-26-oyl-CoA + acceptor = 3-oxo-cholest-4,24-dien-26-oyl-CoA + reduced acceptor. UniProt ... cholest-4,24-dien-26-oyl-CoA (PubMed:26348625, PubMed:26161441). Also able to dehydrogenate steroyl-CoA such as 3-oxo-chol-4-en ...

http://www.rcsb.org/pdb/protein/I6YCA3

Compare acyl-CoA dehydrogenase family member 9 ELISA Kits from leading suppliers on Biocompare. View specifications, prices, ... acyl-CoA dehydrogenase family member 9 ELISA Kits. The ELISA (enzyme-linked immunosorbent assay) is a widely used application ... Your search returned 13 acyl-CoA dehydrogenase family member 9 ELISA ELISA Kit across 2 suppliers. ...

https://www.biocompare.com/pfu/110627/soids/2-2273439/ELISA_Kit/ELISA_acyl-CoA_dehydrogenase_family_member_9

Acyl CoA dehydrogenase is the enzyme used to catalyze the first step of β-oxidation in Fatty acid metabolism. ... Medium-chain acyl-coenzyme A dehydrogenase deficiency ("MCAD") ACADS. C-2 to C-3 short chain. Short-chain acyl-coenzyme A ... Acyl-CoA+Dehydrogenase at the US National Library of Medicine Medical Subject Headings (MeSH) ... Retrieved from "https://www.wikidoc.org/index.php?title=Acyl_CoA_dehydrogenase&oldid=264418" ...

http://wikidoc.org/index.php?title=Acyl_CoA_dehydrogenase&printable=yes

Short-chain specific acyl-CoA dehydrogenase, mitochondrial. Details. Name. Short-chain specific acyl-CoA dehydrogenase, ... Short-chain specific acyl-CoA dehydrogenase, mitochondrial. P16219. Details. Drug Relations. Drug Relations. DrugBank ID. Name ...

https://www.drugbank.ca/bio_entities/BE0000762

2 patients with very long chain Acyl-CoA dehydrogenase deficiency experience fatigue, insomnia, depressed mood, pain, and ... Find the most comprehensive real-world symptom and treatment data on very long chain Acyl-CoA dehydrogenase deficiency at ... What is very long chain Acyl-CoA dehydrogenase deficiency?. Very long chain acyl-coenzyme A dehydrogenase deficiency is a ... Very long chain Acyl-CoA dehydrogenase deficiency Were all in this for good.. ...

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

What is acyl-CoA dehydrogenases? Meaning of acyl-CoA dehydrogenases medical term. What does acyl-CoA dehydrogenases mean? ... Looking for online definition of acyl-CoA dehydrogenases in the Medical Dictionary? acyl-CoA dehydrogenases explanation free. ... acyl-CoA dehydrogenases. acyl-CoA dehydrogenases. Enzymes that activate the first stage of the oxidation of fatty acids.. ... Selective Inhibition of Acyl-CoA Dehydrogenases by a Metabolite of Hypoglycin.. Inactivation of General Acyl-CoA Dehydrogenase ...

http://medical-dictionary.thefreedictionary.com/acyl-CoA+dehydrogenases

Multiple acyl-CoA dehydrogenase deficiency (MADD) is a life-threatening, ultrarare inborn error of metabolism. Case reports ... Multiple acyl-CoA dehydrogenase deficiency (MADD; also known as glutaric aciduria type II, OMIM 231680) is an ultrarare (i.e ... Efficacy and safety of D,L-3-hydroxybutyrate (D,L-3-HB) treatment in multiple acyl-CoA dehydrogenase deficiency. *Willemijn J. ... Multiple acyl-CoA dehydrogenase deficiency (MADD) is a life-threatening, ultrarare inborn error of metabolism. Case reports ...

https://www.nature.com/articles/s41436-019-0739-z?error=cookies_not_supported&code=ac0b4245-21ee-4d67-bdec-bda5543da3a8

3-OPDC-CoA). Also able to dehydrogenate steroyl-CoA such as 3-oxo-chol-4-en-24-oyl-CoA (3-OCO-CoA), 1beta-(2-propanoyl-CoA)-3a ... propanoyl-CoA ester side chains of 3-oxo-4-pregnene-20-carboxyl-CoA (3-OPC-CoA) to yield 3-oxo-4,17-pregnadiene-20-carboxyl-CoA ... H-7a-beta-methylhexahydro-4-indanone (indanone-CoA ester), hexahydroindanone and pregenenone (PubMed:22045806, PubMed:23560677 ... IPR009100 AcylCoA_DH/oxidase_NM_dom. Pfami. View protein in Pfam. PF00441 Acyl-CoA_dh_1, 1 hit. PF02771 Acyl-CoA_dh_N, 1 hit ...

https://www.uniprot.org/uniprot/P71857

Handbook of Genetic Counseling/Short Chain Acyl-CoA Dehydrogenase (SCAD). From Wikibooks, open books for an open world ... lab results: skeletal muscle carnitine low, deficiency of skeletal muscle mitochondrial short-chain acyl-CoA (butyryl-CoA) ... during which time the long and medium acyl-CoA dehydrogenases are unable to compensate for the lack of SCAD ... Retrieved from "https://en.wikibooks.org/w/index.php?title=Handbook_of_Genetic_Counseling/Short_Chain_Acyl-CoA_Dehydrogenase_( ...

https://en.wikibooks.org/wiki/Handbook_of_Genetic_Counseling/Short_Chain_Acyl-CoA_Dehydrogenase_

The octanoyl-CoA oxidation rate, therefore, allows a risk assessment at birth and the identification of new ACADM genotypes ... This demonstrates a correlation between the octanoyl-CoA oxidation rate in lymphocytes and the clinical outcome. With newborn ... activities as measured by octanoyl-CoA oxidation in lymphocytes with both genotype and relevant medical reports in 65 newborns ... Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (OMIM 201450) is the most common inherited disorder of fatty acid ...

http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0045110

Very long-chain-specific acyl-CoA dehydrogenase, mitochondrialImported. ,p>Information which has been imported from another ... tr,J3QKU9,J3QKU9_HUMAN Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial (Fragment) OS=Homo sapiens OX=9606 GN= ... Very long-chain specific acyl-CoA dehydrogenase, mitochondrial, VLCAD, EC 1.3.8.9 ... IPR013786, AcylCoA_DH/ox_N. IPR037069, AcylCoA_DH/ox_N_sf. IPR009100, AcylCoA_DH/oxidase_NM_dom. ...

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

Acyl-CoA dehydrogenases catalyze the alpha,beta-dehydrogenation of acyl-CoA thioesters to the corresponding trans 2,3-eno… ... Mammalian Co-A dehydrogenases (EC:1.3.99.3) are enzymes that catalyse the first step in each cycle of beta-oxidation in ...

http://www.nextbio.com/b/search/ov/Acyl-CoA%20dehydrogenase%2C%20N-terminal

Late-onset multiple acyl-CoA dehydrogenase deficiency (MADD) with electron transfer flavoprotein dehydrogenase (ETFDH) gene ... Multiple Acyl Coenzyme A Dehydrogenase Deficiency/diagnostic imaging. *Multiple Acyl Coenzyme A Dehydrogenase Deficiency/ ... Multiple Acyl Coenzyme A Dehydrogenase Deficiency/metabolism. *Multiple Acyl Coenzyme A Dehydrogenase Deficiency/ ... Clinical heterogeneity; ETFDH; Late-onset multiple acyl-CoA dehydrogenase deficiency; Lipid storage myopathy; Variants ...

https://www.ncbi.nlm.nih.gov/pubmed/27000805

What is acyl-CoA dehydrogenase? Meaning of acyl-CoA dehydrogenase medical term. What does acyl-CoA dehydrogenase mean? ... Looking for online definition of acyl-CoA dehydrogenase in the Medical Dictionary? acyl-CoA dehydrogenase explanation free. ... acyl-CoA dehydrogenase. acyl-CoA dehydrogenase. /ac·yl-CoA de·hy·dro·gen·ase/ (de-hi´dro-jen-ās) any of several enzymes that ... long-chain acyl-CoA dehydrogenase (ACADL), acyl-CoA synthetase (ACSL1), very long chain acyl-CoA dehydrogenase (ACADVL), ...

http://medical-dictionary.thefreedictionary.com/acyl-CoA+dehydrogenase

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common disorder associated with fatty acid oxidation. The ... Screening for medium-chain acyl CoA dehydrogenase deficiency: current perspectives Claudia Soler-Alfonso,1 Michael J Bennett,2 ... Medium chain acyl-CoA dehydrogenase deficiency caused by a deletion of exons 11 and 12. Hum Mol Genet. 1995;4(4):747-749. ... Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: diagnosis by acylcarnitine analysis in blood. Am J Hum Genet. 1993;52(5 ...

https://www.dovepress.com/screening-for-medium-chain-acyl-coa-dehydrogenase-deficiency-current-p-peer-reviewed-fulltext-article-RRN

N2 - PURPOSE: To evaluate the Dutch newborn screening (NBS) for Medium-Chain Acyl-CoA Dehydrogenase (MCAD) deficiency since ... AB - PURPOSE: To evaluate the Dutch newborn screening (NBS) for Medium-Chain Acyl-CoA Dehydrogenase (MCAD) deficiency since ... abstract = "PURPOSE: To evaluate the Dutch newborn screening (NBS) for Medium-Chain Acyl-CoA Dehydrogenase (MCAD) deficiency ... A Nationwide Retrospective Observational Study Of Population Newborn Screening For Medium-Chain Acyl-CoA Dehydrogenase (MCAD) ...

https://www.rug.nl/research/portal/publications/a-nationwide-retrospective-observational-study-of-population-newborn-screening-for-mediumchain-acylcoa-dehydrogenase-mcad-deficiency-in-the-netherlands

... ... is the substrate for histone butyrylation and its abundance is regulated by acyl-CoA dehydrogenase short chain (ACADS). The ... Thus, we hypothesized that the fatty acid intermediate, butyryl-CoA, ... signifying that fatty acid synthesis from carbohydrates substitutes for dietary fat as a source of butyryl-CoA. A high-fat diet ...

https://pubmed.ncbi.nlm.nih.gov/33989779/?utm_source=WordPress&utm_medium=rss&utm_campaign=None&utm_content=1nASi37tjavVgwsXmk5YUf_GRBPtoGVcaaaxKnz2ejNfc5MMoY&fc=None&ff=20210517045118&v=2.14.4

77 Mutations of Human Medium-Chain Acyl-CoA Dehydrogenase. SZABOLCS UDVARI, PETER BROSS, BRAGE S ANDRESEN, NIELS GREGERSEN, ... 77 Mutations of Human Medium-Chain Acyl-CoA Dehydrogenase. SZABOLCS UDVARI, PETER BROSS, BRAGE S ANDRESEN, NIELS GREGERSEN, ... 77 Mutations of Human Medium-Chain Acyl-CoA Dehydrogenase Message Subject (Your Name) has forwarded a page to you from ...

http://www.biochemsoctrans.org/content/26/1/S65

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

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

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

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

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

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

"Acyl-CoA Dehydrogenase, Long-Chain" by people in this website by year, and whether "Acyl-CoA Dehydrogenase, Long-Chain" was a ... "Acyl-CoA Dehydrogenase, Long-Chain" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH ... Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy. Mol Ther. 2012 Jun; ... A near-miss: very long chain acyl-CoA dehydrogenase deficiency with normal primary markers in the initial well-timed newborn ...

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

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a condition that prevents the body from converting certain fats to energy, particularly during periods without food (fasting). (medlineplus.gov)
The natural history of medium-chain acyl CoA dehydrogenase deficiency in the Netherlands: clinical presentation and outcome. (medlineplus.gov)
Dezateux C. Newborn screening for medium chain acyl-CoA dehydrogenase deficiency: evaluating the effects on outcome. (medlineplus.gov)
Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening. (medlineplus.gov)
Joy P, Black C, Rocca A, Haas M, Wilcken B. Neuropsychological functioning in children with medium chain acyl coenzyme a dehydrogenase deficiency (MCADD): the impact of early diagnosis and screening on outcome. (medlineplus.gov)
Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California. (medlineplus.gov)
Jethva R, Bennett MJ, Vockley J. Short-chain acyl-coenzyme A dehydrogenase deficiency. (medlineplus.gov)
The most common fatty acid oxidation disorder, medium chain acyl-CoA dehydrogenase deficiency (MCADD), has become the focal point for the adoption of tandem mass spectrometry to detect it and related inborn errors of metabolism. (cdc.gov)
Despite the increase in screening, understanding of the nat dehydrogenase deficiency" or "tandem mass spectrometry" to ural history of unscreened MCADD remains limited.1,3,4 gether with "newborn screening. (cdc.gov)
Grunert SC (2014) Clinical and genetical heterogeneity of late-onset multiple acyl-coenzyme A dehydrogenase deficiency. (springer.com)
Antozzi C, Garavaglia B, Mora M, Rimoldi M, Morandi L, Ursino E, DiDonato S (1994) Late-onset riboflavin-responsive myopathy with combined multiple acyl coenzyme A dehydrogenase and respiratory chain deficiency. (springer.com)
Olsen RK, Andresen BS, Christensen E, Bross P, Skovby F, Gregersen N (2003) Clear relationship between ETF/ETFDH genotype and phenotype in patients with multiple acyl-CoA dehydrogenation deficiency. (springer.com)
One potential hotspot ACADVL mutation in Chinese patients with very-long-chain acyl-coenzyme A dehydrogenase deficiency. (nih.gov)
Analysis of ACADVL gene variations among nine neonates with very long chain acyl-coA dehydrogenase deficiency]. (nih.gov)
The diagnostic challenge in very-long chain acyl-CoA dehydrogenase deficiency (VLCADD). (nih.gov)
Sequencing of the ACADVL gene revealed that all individuals with activities below 24% were true Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) patients, individuals with residual activities between 24 and 27% carried either one or two mutations. (nih.gov)
When you share what it's like to have very long chain Acyl-CoA dehydrogenase deficiency through your profile, those stories and data appear here too. (patientslikeme.com)
Got a question about living with very long chain Acyl-CoA dehydrogenase deficiency? (patientslikeme.com)
Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: iden tification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism. (thefreedictionary.com)
As a newborn-screening assay, MS/MS is not merely a method for detecting medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (7), nor is it simply an improved method for accurate neonatal detection of PKU with a false-positive rate 10-fold lower than the best method previously available (8). (thefreedictionary.com)
Multiple acyl-CoA dehydrogenase deficiency (MADD) is a life-threatening, ultrarare inborn error of metabolism. (nature.com)
Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. (wikibooks.org)
Significant clinical heterogeneity with similar ETFDH genotype in three Chinese patients with late-onset multiple acyl-CoA dehydrogenase deficiency. (nih.gov)
Late-onset multiple acyl-CoA dehydrogenase deficiency (MADD) with electron transfer flavoprotein dehydrogenase (ETFDH) gene mutations is the most common lipid storage myopathy (LSM) in China. (nih.gov)
Background: Clinically, it is difficult to differentiate multiple acyl-CoA dehydrogenase deficiency (MADD) from immune-mediated necrotizing myopathy (IMNM) because they display similar symptoms. (thefreedictionary.com)
As Max discovers, he has medium chain acyl-CoA dehydrogenase deficiency, or MCADD. (thefreedictionary.com)
This infant has an autosomal recessive disorder described as multiple acyl-CoA dehydrogenase deficiency (MADD). (thefreedictionary.com)
Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency. (thefreedictionary.com)
Conditions currently screened for in Wales are congenital hypothyroidism, cystic fibrosis, medium chain acyl-CoA dehydrogenase deficiency (MCADD), phenylketonuria and sickle cell disorders. (thefreedictionary.com)
Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (MIM 201475) was first described in 1993. (thefreedictionary.com)
We were able to save about 15 babies with medium-chain acyl-CoA dehydrogenase or MCAD deficiency, a condition that prevents the body from converting certain fats in food to energy," Dr al-Rifai said, adding: "The early detection has allowed for early treatment. (thefreedictionary.com)
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common disorder associated with fatty acid oxidation. (dovepress.com)
abstract = "PURPOSE: To evaluate the Dutch newborn screening (NBS) for Medium-Chain Acyl-CoA Dehydrogenase (MCAD) deficiency since 2007.METHODS: A nationwide retrospective, observational study of clinical, laboratory and epidemiological parameters of patients with MCAD deficiency born between 2007-2015. (rug.nl)
A novel tandem mass spectrometry method for rapid confirmation of medium- and very long-chain acyl-CoA dehydrogenase deficiency in newborns. (labome.org)
This clear genotype-phenotype relationship is in sharp contrast to what has been observed in medium-chain acyl-CoA dehydrogenase deficiency, in which no correlation between genotype and phenotype can be established. (nih.gov)
Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy. (umassmed.edu)
A near-miss: very long chain acyl-CoA dehydrogenase deficiency with normal primary markers in the initial well-timed newborn screening specimen. (umassmed.edu)
Spiekerkoetter U, Sun B, Zytkovicz T, Wanders R, Strauss AW, Wendel U. MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency. (umassmed.edu)
49 Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is a rare genetic condition that prevents the body from converting certain fats (called short-chain fatty acids) into energy. (malacards.org)
These mutations lead to a shortage (deficiency) of an enzyme known as short-chain acyl-CoA dehydrogenase, which is involved in the breakdown of short-chain fatty acids. (malacards.org)
Acyl-Coa Dehydrogenase, Short-Chain, Deficiency of, also known as scad deficiency , is related to acyl-coa dehydrogenase, very long-chain, deficiency of and myopathy , and has symptoms including seizures , lethargy and failure to thrive . (malacards.org)
An important gene associated with Acyl-Coa Dehydrogenase, Short-Chain, Deficiency of is ACADS (Acyl-CoA Dehydrogenase Short Chain), and among its related pathways/superpathways are Fatty acid degradation and Fatty acid metabolism . (malacards.org)
71 Acyl-CoA dehydrogenase short-chain deficiency: An inborn error of mitochondrial fatty acid beta-oxidation resulting in acute acidosis and muscle weakness in infants, and a form of lipid- storage myopathy in adults. (malacards.org)
A patient with very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is reported. (bmj.com)
Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a recently identified inborn error of a membrane bound mitochondrial enzyme. (bmj.com)
Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare, inherited (genetic) disease. (newbornscreening.on.ca)
Molecular test to confirm a diagnosis or identify carriers of medium chain acyl-CoA dehydrogenase (MCAD) deficiency for individuals with suggestive clinical and/or biochemical findings. (aruplab.com)
Acyl-Coa Dehydrogenase Deficiency is related to multiple acyl-coa dehydrogenase deficiency, severe neonatal type and multiple acyl-coa dehydrogenase deficiency, mild type . (malacards.org)
An important gene associated with Acyl-Coa Dehydrogenase Deficiency is ACADSB (Acyl-CoA Dehydrogenase Short/Branched Chain), and among its related pathways/superpathways are Metabolism and Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha) . (malacards.org)
A genetic disorder characterized by deficiency of the enzyme medium-chain acyl-coenzyme a dehydrogenase that metabolizes medium-chain fatty acids. (icd10data.com)
Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare genetic condition resulting from a mutation (change) in a person's DNA. (diseaseinfosearch.org)
Following organizations serve the condition "Very long chain acyl-CoA dehydrogenase deficiency" for support, advocacy or research. (diseaseinfosearch.org)
Clinical symptoms are consistent with acquired multiple acyl-CoA dehydrogenase deficiency (MADD). (usda.gov)
Recurrent ACADVL molecular findings in individuals with a positive newborn screen for very long chain acyl-coA dehydrogenase (VLCAD) deficiency in the United States. (semanticscholar.org)
Very long chain acyl-coA dehydrogenase deficiency (VLCADD) is an autosomal recessive inborn error of fatty acid oxidation detected by newborn screening (NBS). (semanticscholar.org)
Infants suspected to have very-long chain acyl-CoA dehydrogenase deficiency from newborn screening. (semanticscholar.org)
Positive newborn screen in a normal infant of a mother with asymptomatic very long-chain Acyl-CoA dehydrogenase deficiency. (semanticscholar.org)
Deficiency of 2-methylbutyryl-CoA dehydrogenase results in the inability to break down isoleucine causing an organic acidemia. (medicalhomeportal.org)
Short/Branched Chain Acyl-CoA Dehydrogenase Deficiency - Information for Professionals (STAR-G) ] More and more cases have been identified by neonatal screening, but they are not published given the benign clinical course. (medicalhomeportal.org)
Educate the family regarding signs, symptoms, and the need for urgent care when the infant becomes ill (see 2-Methylbutyryl CoA Dehydrogenase Deficiency - Information for Parents (STAR-G) for additional information). (medicalhomeportal.org)
Because short-chain acyl-CoA dehydrogenase deficiency (SCADD) (the result of an intramitochondrial defect in the beta-oxidation of fatty acids) may impair this form of energy production, metabolic crisis may result. (medicalhomeportal.org)
Educate the family regarding the benign clinical course of this condition (see Short-Chain Acyl-CoA Dehydrogenase Deficiency - Information for Parents (STAR-G) ). (medicalhomeportal.org)
Based on this proposition, the current study addressed the metabolic consequences of short-chain acyl-coenzyme A (CoA) dehydrogenase (SCAD) deficiency and dietary fat content on liver metabolism, including effects on tissue acylcarnitines, mitochondrial oxidative metabolism, hepatic pAMPK level, and genome-wide transcription. (biomedcentral.com)
A heterozygous missense mutation in adolescent-onset very long-chain acyl-CoA dehydrogenase deficiency with exercise-induced rhabdomyolysis. (semanticscholar.org)
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency. (semanticscholar.org)
This infant may have Very Long Chain Acyl-oA Dehydrogenase deficiency. (archildrens.org)
In order to determine which are useful early diagnostic markers for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, we have analysed urine from an asymptomatic neonate. (elsevier.com)
The Invitae Very Long Chain Acyl-CoA Dehydrogenase Deficiency Test analyzes the ACADVL gene, which is associated with very long chain acyl-CoA dehydrogenase ( VLCAD ) deficiency. (invitae.com)
E71.312 is a billable ICD code used to specify a diagnosis of short chain acyl CoA dehydrogenase deficiency. (icd.codes)
Increased antioxidant response in medium-chain acyl-CoA dehydrogenase deficiency: does lipoic acid have a protective role? (regionh.dk)
BACKGROUND: Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (MCADD) is the most frequent fatty acid oxidation (FAO) defect in humans. (regionh.dk)
The symptoms of the short chain acyl co enzyme A dehydrogenase which is referred as the SCADD deficiency are triggered by the periods of fasting or illness which includes the viral infections. (alldiseases.org)
This disorder includes the mutations in ACDAS gene which causes the short chain acyl co enzyme A dehydrogenase deficiency which is referred as the SCADD. (alldiseases.org)
Medium chain acyl-CoA dehydrogenase deficiency human genome epidemiology review. (cdc.gov)
More than 55 mutations in the ACADS gene have been found to cause short-chain acyl-CoA dehydrogenase (SCAD) deficiency. (nih.gov)
Mutations of ACADS gene associated with short-chain acyl-coenzyme A dehydrogenase deficiency. (nih.gov)
Mutations in this gene have been associated with short-chain acyl-CoA dehydrogenase (SCAD) deficiency. (genecards.org)
Population spectrum of ACADM genotypes correlated to biochemical phenotypes in newborn screening for medium-chain acyl-CoA dehydrogenase deficiency. (nih.gov)
Below you will find more information about Acyl-CoA dehydrogenase, very long chain, deficiency of from Medigest. (medigest.uk)
If you believe that you are suffering from any of the symptoms of Acyl-CoA dehydrogenase, very long chain, deficiency of it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. (medigest.uk)
There are medical conditions that carry similar symptoms associated with Acyl-CoA dehydrogenase, very long chain, deficiency of and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. (medigest.uk)
The information relating to Acyl-CoA dehydrogenase, very long chain, deficiency of comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown. (medigest.uk)
Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) is a rare autosomal recessive condition in which the body fails to oxidize fatty acids because an enzyme is either missing or not functioning correctly. (medigest.uk)
A major component of the medical treatment of medium-chain acyl-coenzyme A (CoA) dehydrogenase (MCAD) deficiency is a diet that permits adequate nutrition and avoids any fasting period longer than 4-5 hours. (medscape.com)
Medium-chain acyl-CoA dehydrogenase deficiency in children with non- ketotic hypoglycemia and low carnitine levels. (medscape.com)
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)
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)
Deficiency in ETF dehydrogenase causes the human genetic disease multiple acyl-CoA dehydrogenase deficiency. (wikipedia.org)
Deficiency of ETF-QO results in a disorder known as glutaric acidemia type II (also known as MADD for multiple acyl-CoA dehydrogenase deficiency), in which there is an improper buildup of fats and proteins in the body. (wikipedia.org)
Very-Long-Chain Acyl-CoA Dehydrogenase (VLCAD) deficiency is a disorder of fatty acid oxidation included in the recommended uniform newborn screening (NBS) panel in the USA. (cdc.gov)
Medium-chain acyl-CoA dehydrogenase deficiency is an inherited condition characterized by inadequate levels of an enzyme required to break down medium-chain fatty acids. (nih.gov)
The five most commonly diagnosed conditions in the United States are 1) hearing loss, 2) primary congenital hypothyroidism, 3) cystic fibrosis, 4) sickle cell disease, and 5) medium-chain acyl-CoA dehydrogenase deficiency ( Table ) (3.6). (cdc.gov)
Coates PM, Hale DE, Stanley CA, Corkey BE, Cortner JA , Genetic deficiency of medium-chain acyl coenzyme A dehydrogenase: studies in cultured skin fibroblasts and peripheral mononuclear leukocytes. (coriell.org)
Newborn screening for medium- and very long-chain acyl-CoA dehydrogenase (MCAD and VLCAD, respectively) deficiency, using acylcarnitine profiling with tandem mass spectrometry, has increased the number of patients with fatty acid oxidation disorders due to the identification of additional milder, and so far silent, phenotypes. (cdc.gov)
To investigate the clinical and laboratory features of very long chain acyl-CoA dehydrogenase deficiency ( VLCADD ) and the correlations between its genotype and phenotype . (bvsalud.org)
Medium chain acyl-CoA dehydrogenase deficiency (MCADD) is a disorder of mitochondrial fatty acid oxidation and is one of the most common inborn errors of metabolism. (biomedcentral.com)
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common inherited disorder of the mitochondrial fatty acid oxidation, caused by mutations in the ACADM gene. (biomedcentral.com)
In the current study, the regression analysis was performed to identify short chain acyl-CoA dehydrogenase (SCAD) deficiency, medium chain acyl-CoA dehydrogenase (MCAD) deficiency, and very long chain acyl-CoA dehydrogenase (VLCAD) deficiency. (figshare.com)
Deficiency of very long-chain acyl-CoA dehydrogenase (VLCAD) is the most common disorder of mitochondrial β-oxidation of long-chain fatty acids. (springeropen.com)
Multiple acyl-CoA dehydrogenase deficiency (MADD), previously called glutaric aciduria type II, is a rare congenital metabolic disorder of fatty acids and amino acids oxidation, with recessive autosomal transmission. (biomedcentral.com)

Acyl-CoA dehydrogenases (ACADs) are a class of enzymes that function to catalyze the initial step in each cycle of fatty acid β-oxidation in the mitochondria of cells. (wikipedia.org)
The following reaction is the oxidation of the fatty acid by FAD to afford an α,β-unsaturated fatty acid thioester of Coenzyme A: ACADs can be categorized into three distinct groups based on their specificity for short-, medium-, or long-chain fatty acid acyl-CoA substrates. (wikipedia.org)
While different dehydrogenases target fatty acids of varying chain length, all types of ACADs are mechanistically similar. (wikipedia.org)
The medium chain acyl-CoA dehydrogenase (MCAD) is the best known structure of all ACADs, and is the most commonly deficient enzyme within the class that leads to metabolic disorders in animals. (wikipedia.org)
Thus, we hypothesized that the fatty acid intermediate, butyryl-CoA, is the substrate for histone butyrylation and its abundance is regulated by acyl-CoA dehydrogenase short chain (ACADS). (nih.gov)
We showed previously that mice with genetically inactivated Acads , encoding short-chain acyl-CoA dehydrogenase (SCAD), shift food consumption away from fat and toward carbohydrate when tested in a macronutrient choice paradigm. (biomedcentral.com)
Acads encodes short-chain acyl-CoA dehydrogenase (SCAD), a member of the family of four enzymes that catalyzes the first of four sequential steps in the mitochondrial fatty acid oxidation spiral which produces acetyl-CoA for the tricarboxylic acid cycle. (biomedcentral.com)
Previously we reported that mice with a global, genetic inactivation of the gene encoding short-chain acyl-CoA dehydrogenase ( Acads−/− ) shift consumption away from fat and toward carbohydrate when offered a choice between macronutrient-rich diets [ 1 ]. (biomedcentral.com)
The ACADS gene provides instructions for making an enzyme called short-chain acyl-CoA dehydrogenase (SCAD). (nih.gov)
ACADS (Acyl-CoA Dehydrogenase Short Chain) is a Protein Coding gene. (genecards.org)

MCAD can bind to a rather broad range of chain-lengths in the acyl-CoA substrate, however studies show that its specificity tends to target octanoyl-CoA (C8-CoA). (wikipedia.org)
acyl-CoA dehydrogenase (MCAD) protein. (cdc.gov)
2]) upon reduction with its substrate, medium chain acyl-CoA dehydrogenase (MCAD). (thefreedictionary.com)
We have determined the frequency of the A985G mutation in the medium-chain acyl-CoA dehydrogenase (MCAD) gene in a cohort of 1142 healthy babies born in two Geneva hospitals. (epfl.ch)
Medium chain acyl-CoA dehydrogenase (MCAD) is a tetrameric flavoprotein essential for the beta-oxidation of medium chain fatty acids. (cdc.gov)
The ACADM gene provides instructions for making an enzyme called medium-chain acyl-CoA dehydrogenase (MCAD). (nih.gov)
To discern whether two related nuclear genes are expressed similarly, the tissue distribution and developmental profile of the rat long- and medium-chain acyl-CoA dehydrogenase (LCAD and MCAD) mRNAs were compared. (elsevier.com)
LC-MS/MS was used to measure MCAD- or VLCAD-catalyzed production of enoyl-CoA and hydroxyacyl-CoA, in human lymphocytes. (cdc.gov)
Enzyme analyses with C6-CoA, C6-CoA + C4-CoA, and PP-CoA identified significantly higher residual MCAD enzyme activities in subjects with variant ACADM genotypes when compared to patients with classical ACADM genotypes. (biomedcentral.com)

Short-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 (By similarity). (genecards.org)
Among the different mitochondrial acyl-CoA dehydrogenases, short-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 4 to 6 carbons long primary chains (PubMed:21237683, PubMed:11134486). (genecards.org)

The first step of the [beta]-oxidation cycle is catalyzed by acyl-CoA dehydrogenases , whereas the enzymes of MTP catalyze the last 3 steps of [beta]-oxidation. (thefreedictionary.com)
Additionally, acyl-CoA dehydrogenase , very long chain, which is involved in a fatty acid [beta]-oxidation pathway, was found in the QTL interval on SSC12 [14]. (thefreedictionary.com)
Very-long-chain acyl-CoA dehydrogenase (VLCAD) catalyzes the initial rate-limiting step in mitochondrial fatty acid beta-oxidation. (nih.gov)
Previous analysis of the riboflavin-deficient rat has shown that the failure of fatty acid oxidation is due to a decrease in the activity of the acyl-CoA dehydrogenases of beta-oxidation. (biochemj.org)
Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of four straight-chain acyl-CoA dehydrogenase (ACD) enzymes, which are all nuclear encoded mitochondrial flavoproteins catalyzing the initial step in fatty acid beta-oxidation. (semanticscholar.org)
Without acyl CoA dehydrogenase to initiate the first step of mitochondrial beta-oxidation, your ability to metabolize fats is inhibited (1). (blogspot.com)
Because the body relies on the production of acetyl CoA from its storage of fat for energy, the lack of acetyl CoA dehydrogenase and in its capacity to produce beta-oxidation causes the body to end up in a hypoglycemic (no glucose) and hypoketotic state (no ketones from oxidation of fatty acids) (1p159;2-3). (blogspot.com)
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 (By similarity). (genecards.org)

In isolated muscle mitochondria, cytochromes aa3, b and c were partially decreased, butyryl-CoA dehydrogenase and palmitoyl-CoA dehydrogenase activities were 10 and 54% of the normal, respectively. (elsevier.com)

The optimum substrate for SCAD is butyryl-CoA, a fatty acid with four carbon units. (biomedcentral.com)

This gives a total of four FAD molecules and four acyl-CoA substrate binding sites per enzyme. (wikipedia.org)
The acyl-CoA substrate is bound completely within each monomer of the enzyme. (wikipedia.org)
In enzymology, an acyl-CoA dehydrogenase (NADP+) (EC 1.3.1.8) is an enzyme that catalyzes the chemical reaction acyl-CoA + NADP+ ⇌ {\displaystyle \rightleftharpoons } 2,3-dehydroacyl-CoA + NADPH + H+ Thus, the two substrates of this enzyme are acyl-CoA and NADP+, whereas its 3 products are 2,3-dehydroacyl-CoA, NADPH, and H+. (wikipedia.org)
The systematic name of this enzyme class is acyl-CoA:NADP+ 2-oxidoreductase. (wikipedia.org)
This gene provides instructions for making an enzyme called medium-chain acyl-CoA dehydrogenase, which is required to break down (metabolize) a group of fats called medium-chain fatty acids. (medlineplus.gov)
Acyl CoA dehydrogenase is the enzyme used to catalyze the first step of β-oxidation in Fatty acid metabolism . (wikidoc.org)
Deficiencies have been identified in each of these FAO enzyme forms with the exception of long-chain acyl-CoA dehydrogenase (5). (thefreedictionary.com)
The activity of this enzyme is increased such that the ratio of short-chain acyl-CoA dehydrogenase apoenzyme to holoenzyme does not change. (biochemj.org)
The increased mitochondrial specific activity of short-chain acyl-CoA dehydrogenase during starvation may result from an increased availability of flavin coenzyme or an increase in enzyme catalytic efficiency. (biochemj.org)
The enzyme from pig liver can accept substrates with acyl chain lengths of 4 to 16 carbon atoms, but is most active with C8 to C12 compounds. (creative-enzymes.com)
VLCAD occurs when the body either does not make enough or makes non-working enzyme called very long-chain acyl-CoA dehydrogenase. (diseaseinfosearch.org)
That is, the enzyme-one of four depending on fatty acid chain lengths-catalyzes the formation of the double bond between alpha- and beta-carbons, which then are degraded to two-carbon acetyl CoA units (1p159). (blogspot.com)
This led us to study the pyruvate dehydrogenase complex (PDC), the key enzyme in the glycolysis releasing acetyl-CoA to the TCA cycle. (regionh.dk)
It occurs due to the defects present in the enzyme complex to break the certain group of fatty acids and is known as the short chain acyl co enzyme A dehydrogenase which is referred as the SCADD. (alldiseases.org)
These lead to the lower levels of enzyme known as the short chain acyl co enzyme A dehydrogenase which is referred as the SCADD. (alldiseases.org)
Electron-transferring-flavoprotein dehydrogenase (ETF dehydrogenase or electron transfer flavoprotein-ubiquinone oxidoreductase, EC 1.5.5.1) is an enzyme that transfers electrons from electron-transferring flavoprotein in the mitochondrial matrix, to the ubiquinone pool in the inner mitochondrial membrane. (wikipedia.org)
Co enzyme A (CoA) is a ubiquitous cofactor present in every known organism. (mdpi.com)
exceptions are AtuC/AtuF, which constitute the two subunits of geranyl-CoA carboxylase, the key enzyme of the Atu pathway. (microbiologyresearch.org)
Enzyme analyses were performed in leukocytes with: hexanoyl-CoA (C6-CoA) +/− butyryl-CoA (C4-CoA), and phenylpropionyl-CoA (PP-CoA). (biomedcentral.com)

ACADM, a member of the acyl-CoA dehydrogenase (ACAD) family that comprises 9 known proteins, is involved in the oxidation of medium-chain fatty acids and amino acids (Kim Miura, 2004). (thefreedictionary.com)
Recommended when Medium Chain Acyl-CoA Dehydrogenase (ACADM) 2 Mutations ( 0051205 ) does not identify two causative variants. (aruplab.com)
[email protected]#To investigate the effect of medium-chain acyl-CoA dehydrogenase (ACADM) on invasion and metastasis of breast cancer cells and explore the underlying mechanism. (bvsalud.org)

Recombinant adeno-associated virus-mediated gene delivery of long chain acyl coenzyme A dehydrogenase (LCAD) into LCAD-deficient mice. (umassmed.edu)
Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene. (semanticscholar.org)
Nagan N, Kruckeberg KE, Tauscher AL, Bailey KS, Rinaldo P, Matern D. The frequency of short-chain acyl-CoA dehydrogenase gene variants in the US population and correlation with the C(4)-acylcarnitine concentration in newborn blood spots. (nih.gov)
This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. (genecards.org)
Gene Ontology (GO) annotations related to this gene include flavin adenine dinucleotide binding and fatty-acyl-CoA binding . (genecards.org)
Purified PA1535 gene product revealed high citronellyl-CoA dehydrogenase activity ( V max 2450 mU mg −1 ) but had significantly lower affinity than AtuD to citronellyl-CoA ( K m 18 μM). (microbiologyresearch.org)

Other names in common use include 2-enoyl-CoA reductase, dehydrogenase, acyl coenzyme A (nicotinamide adenine dinucleotide, phosphate), enoyl coenzyme A reductase, crotonyl coenzyme A reductase, crotonyl-CoA reductase, and acyl-CoA dehydrogenase (NADP+). (wikipedia.org)
This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. (nih.gov)
ac·yl-CoA de·hy·dro·gen·ase/ ( de-hi´dro-jen-ās ) any of several enzymes that catalyze the oxidation of acyl coenzyme A thioesters as a step in the degradation of fatty acids. (thefreedictionary.com)
Long-chain 3-hydroxy acyl-coenzyme A dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that comprise the trifunctional protein of the inner mitochondrial membrane. (medigest.uk)
The other 2 activities of the protein are long-chain 3-ketoacyl CoA thiolase (LCKT) and 2-enoyl coenzyme A (CoA) hydratase (LCEH). (medigest.uk)
The mitochondrial isovaleryl-coenzyme A dehydrogenase of Arabidopsis oxidizes intermediates of leucine and valine catabolism. (microbiologyresearch.org)

Medium-chain acyl-CoAs generated by long-chain specific enzymes at the inner mitochondrial membrane undergo a series of enzymatic steps in the matrix to generate acetyl-CoA and a chain-shortened acyl-CoA. (dovepress.com)
In vitro studies were performed using isovaleryl-CoA dehydrogenase (IVD), isobutyryl-CoA dehydrogenase (IBD) and short branched-chain acyl-CoA dehydrogenase (SBCAD), enzymes involved in the degradation pathway of leucine, valine and isoleucine. (aspetjournals.org)
The enzymatic activities of the three purified human enzymes were measured using optimized HPLC procedures and the respective kinetic parameters were determined in the absence and presence of VPA and the corresponding CoA and dephosphoCoA conjugates. (aspetjournals.org)
In the present work, we sought to compare the dehydrogenase activities and H2O2-producing capacities of VLCAD and LCAD using isolated liver mitochondria, recombinant enzymes, and the human hepatic cell line HepG2. (informnetwork.org)
Synthesis of CoA-thioesters is vital for the study of CoA-dependent enzymes and pathways, but also as standards for metabolomics studies. (mdpi.com)

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

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

Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress. (labome.org)
Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. (labome.org)
This reduces malonyl-CoA content and promotes mitochondrial fatty acid oxidation by diminishing the inhibitory effect of malonyl CoA on carnitine palmitoyltransferase-1. (diabetesjournals.org)

Pig kidney general acyl-CoA dehydrogenase is markedly stabilized against loss of flavin and activity in 7.3 M-urea or at 60 degrees C upon reduction with sodium dithionite or octanoyl-CoA. (biochemj.org)
cDNA clone for general acyl CoA dehydrogenase (GAD) was isolated from a rat liver cDNA expression library in λgtll using anti-pig kidney GAD antibody. (uni-konstanz.de)

Normally, electrons generated by fatty acids oxidation in the mitochondrial matrix are first transferred to ETF and then electron transfer flavoprotein dehydrogenase (ETFDH = ETF-Ubiquinone-oxidoreductase = ETF-QO). (biomedcentral.com)

In my own specialist area of lipid metabolism, deficiencies of the fatty acid metabolizing acyl-CoA dehydrogenases are defined as a group of diseases that may be potentially treatable with a combination of dietary manipulation and prevention of fasting, because these are essentially diseases of fasting intolerance. (thefreedictionary.com)
Gregersen N, Andresen BS, Corydon MJ, Corydon TJ, Olsen RK, Bolund L, Bross P. Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship. (nih.gov)
Some success in identifying acyl-CoA dehydrogenase (ACAD) deficiencies before they are symptomatic has been achieved through tandem mass spectrometry. (figshare.com)

One of these genes, lipB , encodes an acyl-CoA dehydrogenase homologue. (microbiologyresearch.org)
We measured the oxidation rate of palmitoyl-CoA (C16-CoA) as well as the expression of genes involved in lipogenesis and renal failure. (springeropen.com)

This probably allowed for the substrate binding site to open up considerably to accommodate much larger polycyclic-CoA substrates, rather than fatty acids of varying chain lengths. (wikipedia.org)
Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. (nih.gov)

Most acyl-CoA dehydrogenases are α4 homotetramers, and in two cases (for very long chain fatty acid substrates) they are α2 homodimers. (wikipedia.org)
This is in contrast to the situation for human very long-chain, long-chain, and medium-chain acyl-CoA dehydrogenases , which are stereospecific for the S-enantiomers only of their methyl branched-chain substrates (24). (thefreedictionary.com)
Evidence for impaired gluconeogenesis in very long-chain acyl-CoA dehydrogenase-deficient mice. (labome.org)
ter Veld F, Primassin S, Hoffmann L, Mayatepek E, Spiekerkoetter U. Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice. (labome.org)
Acyl-CoA Dehydrogenase, Long-Chain" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (umassmed.edu)
This graph shows the total number of publications written about "Acyl-CoA Dehydrogenase, Long-Chain" by people in this website by year, and whether "Acyl-CoA Dehydrogenase, Long-Chain" was a major or minor topic of these publications. (umassmed.edu)
Below are the most recent publications written about "Acyl-CoA Dehydrogenase, Long-Chain" by people in Profiles. (umassmed.edu)
EC 1.3.8.1, short-chain acyl-CoA dehydrogenase, EC 1.3.8.8, long-chain acyl-CoA dehydrogenase, and EC 1.3.8.9, very-long-chain acyl-CoA dehydrogenase. (creative-enzymes.com)
Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. (nih.gov)
Long-chain-acyl-CoA dehydrogenase (LCADH) has been produced by recombinant techniques from the human cDNA and purified after expression in Escherichia coli. (uni-konstanz.de)
Recently, very long-chain acyl-CoA dehydrogenase (VLCAD) was shown to have partial oxidase activity and to produce H2O2 in the livers of mice maintained on a high-fat diet. (informnetwork.org)
Rodents are also known to highly express long-chain acyl-CoA dehydrogenase (LCAD), another flavoprotein that functionally overlaps with VLCAD. (informnetwork.org)
Thus, the clinical features may arise from either toxicity due to long-chain acyl-CoA esters that cause cardiomyopathy and cardiac arrhythmias or from a block in long-chain fatty acid oxidation that leads to an inability to synthesize ketone bodies and/or adenosine triphosphate from long-chain fatty acids. (medigest.uk)
The CoA esters produced range from short- to long-chain, include branched and α,β-unsaturated representatives as well as other functional groups. (mdpi.com)
Identification of the catalytic base in long chain acyl-CoA dehydrogenase. (microbiologyresearch.org)

The starvation-associated increase in fatty acid oxidation is mediated by an increase in the mitochondrial short-chain acyl-CoA dehydrogenase activity. (biochemj.org)
We conclude that short-chain acyl-CoA dehydrogenase activity is limiting for fatty acid oxidation when its activity falls below a critical point. (biochemj.org)
Pig kidney LCADH was purified using an optimized method which also produces apparently pure short-chain-acyl-CoA dehydrogenase (SCADH) and medium-chain-acyl-CoA dehydrogenase (MCADH) in good yields. (uni-konstanz.de)
Schmidt SP, Corydon TJ, Pedersen CB, Bross P, Gregersen N. Misfolding of short-chain acyl-CoA dehydrogenase leads to mitochondrial fission and oxidative stress. (nih.gov)

This is the first study demonstrating that acylcarnitines and acyl-CoA directly correlate and concomitantly increase after exercise in VLCAD-deficient muscle. (labome.org)
In vitro, recombinant mouse LCAD (mLCAD) exhibited stronger dehydrogenase activity than mouse VLCAD (mVLCAD) with palmitoyl-CoA as substrate, and produced H2O2 at a significantly higher rate. (informnetwork.org)
To address this question, we measured the palmitoyl-CoA (C16-CoA) oxidation rate in the kidney of wild-type (WT) and VLCAD −/− mice in situations of normal and increased energy demand as well as the expression of other dehydrogenases with overlapping substrate specificity. (springeropen.com)

Their action results in the introduction of a trans double-bond between C2 (α) and C3 (β) of the acyl-CoA thioester substrate. (wikipedia.org)
Based on our results we provide a general guideline to the optimal synthesis method of a given CoA-thioester in respect to its functional group(s) and the commercial availability of the precursor molecule. (mdpi.com)

This class of ACAD was demonstrated to form α2β2 heterotetramers, rather than the usual α4 homotetramer, a protein architecture that evolved in order to accommodate a much larger steroid-CoA substrate. (wikipedia.org)

Acyl-CoA dehydrogenase activity in the riboflavin-deficient rat. (biochemj.org)
C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects. (medscape.com)

This enzyme's action represents the first step in fatty acid metabolism (the process of breaking long chains of fatty acids into acetyl CoA molecules). (wikipedia.org)
On binding to its receptor, gAcrp30 activates AMPK, leading to subsequent inhibition of acetyl-CoA carboxylase ( 1 , 10 ). (diabetesjournals.org)

The thioesters of CoA are core intermediates in many metabolic processes, such as the citric acid cycle, fatty acid biosynthesis and secondary metabolism, including polyketide biosynthesis. (mdpi.com)

Our results demonstrate that LCADH is as important as the other acyl-CoA dehydrogenases in fatty acid oxidation at physiological, mitochondrial pH with optimal substrates of chain length C10 C14. (uni-konstanz.de)

To our knowledge AtuD is the first acyl-CoA dehydrogenase with a documented substrate specificity for terpenoid molecule structure and is essential for a functional Atu pathway. (microbiologyresearch.org)

Friulimicin consists of a cyclic peptide core of ten amino acids and an acyl residue linked to an exocyclic amino acid. (microbiologyresearch.org)

In this study, we provide the first identification and characterization of an L-2,3-BD dehydrogenase from K. pneumoniae, demonstrating its reductive activities toward diacetyl and acetoin, and oxidative activity toward L-2,3-BD. (usda.gov)

Anatomy3

Organisms1

Diseases1

Chemicals and Drugs75

Phenomena and Processes7

Information Science2

Molecular cloning of cDNA encoding mitochondrial very-long-chain acyl-CoA dehydrogenase from bovine heart. (1/257)

The medium-/long-chain fatty acyl-CoA dehydrogenase (fadF) gene of Salmonella typhimurium is a phase 1 starvation-stress response (SSR) locus. (2/257)

Oxidation of medium-chain acyl-CoA esters by extracts of Aspergillus niger: enzymology and characterization of intermediates by HPLC. (3/257)

Outcome of medium chain acyl-CoA dehydrogenase deficiency after diagnosis. (4/257)

A novel acyl-CoA oxidase that can oxidize short-chain acyl-CoA in plant peroxisomes. (5/257)

Cloning and mapping of three pig acyl-CoA dehydrogenase genes. (6/257)

Peroxisome proliferator-activated receptor alpha mediates the adaptive response to fasting. (7/257)

Evaluating newborn screening programmes based on dried blood spots: future challenges. (8/257)

3-Hydroxyacyl CoA Dehydrogenases

Acyl-CoA Dehydrogenases

Acyl-CoA Dehydrogenase

Acyl Coenzyme A

Diacylglycerol O-Acyltransferase

Sterol O-Acyltransferase

L-Lactate Dehydrogenase

Acyltransferases

Alcohol Dehydrogenase

Coenzyme A

Glyceraldehyde-3-Phosphate Dehydrogenases

Coenzyme A Ligases

Aldehyde Dehydrogenase

Glutamate Dehydrogenase

Glucosephosphate Dehydrogenase

Malate Dehydrogenase

Isocitrate Dehydrogenase

Alcohol Oxidoreductases

Acyl-CoA Oxidase

Dihydrolipoamide Dehydrogenase

Carbohydrate Dehydrogenases

Diazepam Binding Inhibitor

Succinate Dehydrogenase

L-Iditol 2-Dehydrogenase

Glycerolphosphate Dehydrogenase

Fatty Acids

NAD

Oxidoreductases

Kinetics

Aldehyde Oxidoreductases

Glucose 1-Dehydrogenase

Hydroxysteroid Dehydrogenases

Ketoglutarate Dehydrogenase Complex

Triazenes

Palmitoyl Coenzyme A

Sugar Alcohol Dehydrogenases

Liver

Glucose Dehydrogenases

3-Hydroxysteroid Dehydrogenases

Molecular Sequence Data

Phosphogluconate Dehydrogenase

NADH Dehydrogenase

IMP Dehydrogenase

Lactate Dehydrogenases

Cholesterol Esters

Formate Dehydrogenases

Amino Acid Sequence

17-Hydroxysteroid Dehydrogenases

Oxidation-Reduction

Xanthine Dehydrogenase

Esterification

Microsomes

Hydroxybutyrate Dehydrogenase

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)

Pyruvate Dehydrogenase (Lipoamide)

Ketone Oxidoreductases

NADP

11-beta-Hydroxysteroid Dehydrogenases

Dihydrouracil Dehydrogenase (NADP)

Uridine Diphosphate Glucose Dehydrogenase

Palmitic Acids

Substrate Specificity

Butyryl-CoA Dehydrogenase

Glucosephosphate Dehydrogenase Deficiency

Lipid Metabolism

Triglycerides

Oleic Acids

Cholesterol

11-beta-Hydroxysteroid Dehydrogenase Type 1

Alanine Dehydrogenase

3-alpha-Hydroxysteroid Dehydrogenase (B-Specific)

Mannitol Dehydrogenases

Microbodies

Carnitine O-Palmitoyltransferase

Hydroxyprostaglandin Dehydrogenases

Glyceraldehyde 3-Phosphate Dehydrogenase (NADP+)

Acyl-CoA Dehydrogenase, Long-Chain

Retinal Dehydrogenase

Acetyl Coenzyme A

Acylation

$Gentaur Molecular :ATGen \ ACADM, 26 421aa, Human, His tag, E.coli \ ATGP0603$ Gentaur Molecular :ATGen \ ACADM, 26 421aa, Human, His tag, E.coli \ ATGP0603