3-Hydroxyacyl CoA Dehydrogenases
Acyl-CoA Dehydrogenase
Acyl Coenzyme A
Diacylglycerol O-Acyltransferase
Sterol O-Acyltransferase
L-Lactate Dehydrogenase
Acyltransferases
Alcohol Dehydrogenase
Glyceraldehyde-3-Phosphate Dehydrogenases
Aldehyde Dehydrogenase
Glutamate Dehydrogenase
Malate Dehydrogenase
Isocitrate Dehydrogenase
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.
Alcohol Oxidoreductases
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).
Acyl-CoA Oxidase
Dihydrolipoamide Dehydrogenase
Carbohydrate Dehydrogenases
Diazepam Binding Inhibitor
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.
Succinate Dehydrogenase
L-Iditol 2-Dehydrogenase
Fatty Acids
NAD
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)
Oxidoreductases
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)
Glucose 1-Dehydrogenase
Hydroxysteroid Dehydrogenases
Triazenes
Palmitoyl Coenzyme A
Sugar Alcohol Dehydrogenases
Liver
Glucose Dehydrogenases
Molecular Sequence Data
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.
Phosphogluconate Dehydrogenase
NADH Dehydrogenase
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.
IMP Dehydrogenase
Cholesterol Esters
Formate Dehydrogenases
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.
Amino Acid Sequence
17-Hydroxysteroid Dehydrogenases
Oxidation-Reduction
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).
Xanthine Dehydrogenase
Esterification
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.
Microsomes
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)
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
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.
Pyruvate Dehydrogenase (Lipoamide)
NADP
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)
11-beta-Hydroxysteroid Dehydrogenases
Dihydrouracil Dehydrogenase (NADP)
Uridine Diphosphate Glucose Dehydrogenase
Substrate Specificity
Butyryl-CoA Dehydrogenase
Glucosephosphate Dehydrogenase Deficiency
Lipid Metabolism
Oleic Acids
Cholesterol
11-beta-Hydroxysteroid Dehydrogenase Type 1
Alanine Dehydrogenase
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.
3-alpha-Hydroxysteroid Dehydrogenase (B-Specific)
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.
Mannitol Dehydrogenases
Microbodies
Carnitine O-Palmitoyltransferase
Hydroxyprostaglandin Dehydrogenases
Acyl-CoA Dehydrogenase, Long-Chain
Retinal Dehydrogenase
Acetyl Coenzyme A
Escherichia coli
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.
20-Hydroxysteroid Dehydrogenases
11-beta-Hydroxysteroid Dehydrogenase Type 2
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.
Lipids
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)
Isovaleryl-CoA Dehydrogenase
Isoenzymes
Multienzyme Complexes
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)
Screening for medium chain acyl-CoA dehydrogenase deficiency using electrospray ionisation tandem mass spectrometry | Archives...
RCSB PDB
- 1EGC: STRUCTURE OF T255E, E376G MUTANT OF HUMAN MEDIUM CHAIN ACYL-COA DEHYDROGENASE COMPLEXED WITH...
RCSB PDB
- 1EGC: STRUCTURE OF T255E, E376G MUTANT OF HUMAN MEDIUM CHAIN ACYL-COA DEHYDROGENASE COMPLEXED WITH...
Functional Effects of Different Medium-Chain Acyl-CoA Dehydrogenase Genotypes and Identification of Asymptomatic Variants
acyl-CoA dehydrogenase deficiency
In vitro and in vivo consequences of variant medium-chain acyl-CoA dehydrogenase genotypes | Orphanet Journal of Rare Diseases ...
77 Mutations of Human Medium-Chain Acyl-CoA Dehydrogenase | Biochemical Society Transactions
Submissions for variant NM 000016.5(ACADM):c.449 452del (p.Thr150fs) (rs786204642) -
ClinVar Miner
High Frequency Carrier Screen (11 Genes) - Sema4
ACADM antibodies | Antibodypedia
Transcriptional control of a nuclear gene encoding a mitochondrial fatty acid oxidation enzyme in transgenic mice: role for...
Short-Chain Acyl-CoA Dehydrogenase Deficiency - ACADS sequencing | The Doctors Laboratory
iMed.ULisboaRita C. Guedes • iMed.ULisboa
DMOZ - Health: Conditions and Diseases: Nutritional and Metabolic Disorders: Cholesterol and Other Fats: Medium Chain Acyl...
Michael J. Bennett, PhD - AACC.org
2018 ICD-10-CM Diagnosis Code E71.311: Medium chain acyl CoA dehydrogenase deficiency
Fibroblast Fatty-Acid Oxidation Flux Assays Stratify Risk in Newborns with Presumptive-Positive Results on Screening for Very...
Altered lipid metabolism in vitamin A deficient liver by Hye Won Kang
3k6p - Proteopedia, life in 3D
Medium-chain acyl-coenzyme A dehydrogenase deficiency - SNPedia
List of variants reported as pathogenic for Medium-chain acyl-coenzyme A dehydrogenase deficiency by Knight Diagnostic...
Newborn Screening Laboratory Bulletin
GM05872
New UCLA study raises questions about genetic testing of newborns | UCLA
My Child Has Mcadd - Circle of Moms
Gentaur Molecular :ATGen \ ACADM, 26 421aa, Human, His tag, E.coli \ ATGP0603
Magazine Review Prior Issues
MCADCafe: DesignShowcase
modelling a cardiovascular stent | MCAD Central
MedLabs Group
Short chain acyl-CoA dehydrogenase deficiency and short-term high-fat diet perturb mitochondrial energy metabolism and...
Acyl CoA dehydrogenase deficiency synonyms, acyl CoA dehydrogenase deficiency antonyms - FreeThesaurus.com
A severe genotype with favourable outcome in very long chain acyl-CoA dehydrogenase deficiency | Archives of Disease in...
Medical Home Portal - Short-Chain Acyl-CoA Dehydrogenase Deficiency (SCADD)
ACADM - Wikipedia
Download Motivation Agency And Public Policy Of Knights And Knaves Pawns And Queens
Short-Chain Acyl-CoA Dehydrogenase (SCAD) Deficiency, Full Gene Analysis - Barnes-Jewish Hospital
Free Energy Surface, Reaction Paths, and Kinetic Isotope Effect of Short-Chain Acyl-CoA Dehydrogenase
Acyl-CoA dehydrogenase, very long chain, deficiency of (Symptoms, signs, causes, treatments & definition) - Medigest
Defective folding and rapid degradation of mutant proteins is a common disease mechanism in genetic disorders<...
Utah Medical Home Portal - Fatty Acid Oxidation Disorders (MCADD, LCHADD, VLCADD)
Fatty acids - Omega 3 as energy sources such as kidney, heart, cerebellum, intestine, and skeletal muscle and nuclear receptor...
Very Long Chain Acyl CoA Dehydrogenase Deficiency (VLCAD) | CHEO NSO
Aging | ACADS acts as a potential methylation biomarker associated with the proliferation and metastasis of hepatocellular...
updike 2017 acdh-1 - MICROPUBLICATION:BIOLOGY
HOGENOMDNA: ALDEN1 2.PE11
IFO 13344 Strain Passport - StrainInfo
Acadvl - Very long-chain-specific acyl-CoA dehydrogenase, mitochondrial - Mus musculus (Mouse) - Acadvl gene & protein
ACADS Pre-design Chimera RNAi - (H00000035-R01) - Products - Abnova
ACAD10 Gene - GeneCards | ACD10 Protein | ACD10 Antibody
LOCUS OA238 5478349 bp DNA circular CON 11-JUN-2013
GeneDx
MCAD Tech News #179 | Cadalyst
Glutaric acidemia type 2
"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-transferring-flavoprotein dehydrogenase. When one of these enzymes is ...
ETFA
"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 ...
ETFB
"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 ...
ACADVL
"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 ...
ACADS
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 ...
Fatty acid
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 ...
Very long chain fatty acid
"Very long-chain acyl-CoA dehydrogenase deficiency". Genetics Home Reference, National Institutes of Health. Retrieved 5 January ...
ACAD10
Acyl-CoA dehydrogenase family, member 10 is a protein that in humans is encoded by the ACAD10 gene. This gene encodes a member ... "Entrez Gene: Acyl-CoA dehydrogenase family, member 10". Bian L, Hanson RL, Muller YL, Ma L, Kobes S, Knowler WC, Bogardus C, ... "Identification and characterization of new long chain acyl-CoA dehydrogenases". Molecular Genetics and Metabolism. 102 (4): 418 ... of the acyl-CoA dehydrogenase family of enzymes (ACADs), which participate in the beta-oxidation of fatty acids in mitochondria ...
Riboflavin-responsive exercise intolerance
Multiple acyl-CoA dehydrogenase deficiency - similar in biochemical features; responsive to riboflavin in the majority of late- ...
Mitochondrial trifunctional protein deficiency
"Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Homo ... "OMIM Entry - * 600890 - HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, ALPHA SUBUNIT; HADHA". omim. ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ...
Fatty acid desaturase
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. ...
ACADM
Wang SS, Fernhoff PM, Hannon WH, Khoury MJ (1999). "Medium chain acyl-CoA dehydrogenase deficiency human genome epidemiology ... "ACADM - Medium-chain specific acyl-CoA dehydrogenase, mitochondrial precursor - Homo sapiens (Human) - ACADM gene & protein". ... "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 ...
ACADSB
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 ...
Nitroalkane oxidase
"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. ...
Oxidative phosphorylation
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 (Articles with short ...
ACADL
... 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 ...
ACAD9
"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- ...
Α,β-Unsaturated carbonyl compound
Two prominent examples are coumaroyl-coenzyme A and crotonyl-coenzyme A. They arise by the action of acyl-CoA dehydrogenases. ... Thorpe C, Kim JJ (June 1995). "Structure and mechanism of action of the acyl-CoA dehydrogenases". FASEB Journal. 9 (9): 718-25 ... it is the acyl group derived from acrylic acid. The preferred IUPAC name for the group is prop-2-enoyl, and it is also known as ...
Mitochondrial folate transporter
The phenotype of the patients is reminiscent of multiple acyl-CoA dehydrogenase deficiency (MADD). According to a review ...
Astrocyte
"Misfolding of short-chain acyl-CoA dehydrogenase leads to mitochondrial fission and oxidative stress". Molecular Genetics and ...
Sodium phenylbutyrate
"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 ...
Hydrogen
"The deuterium isotope effect upon the reaction of fatty acyl-CoA dehydrogenase and butyryl-CoA". J. Biol. Chem. 255 (19): 9093- ...
Methylene cyclopropyl acetic acid
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 ...
Type 2 diabetes
April 2011). "Toxic response caused by a misfolding variant of the mitochondrial protein short-chain acyl-CoA dehydrogenase". ...
Jamaican vomiting sickness
Also, it inhibits acyl-CoA dehydrogenases, so that only unsaturated fatty acids can be fully oxidized. Fatty acids accumulate ...
Newborn screening
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 ...
Spring peeper
These types of males utilize citrate synthase and β-hydroxy acyl CoA dehydrogenase (a protein) in their muscles at greater ...
Erucic acid
... 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. ...
Hydrocarbonoclastic bacteria
The n-alkanals are subsequently transformed into fatty acids and then into acyl CoA, respectively by the aldehyde dehydrogenase ... and by the acyl-CoA synthetase. CH 3 − R − CH 3 ⟶ CH 3 − R − CH 2 OH ⟶ CH 3 − R − CHO ⟶ CH 3 − R − COOH ⟶ ( CH 2 OH ) − R − ... dehydrogenases and others. Furthermore, as they are adapted to grow in hydrocarbon-rich environments, they often synthesize ... n-alkanols are released which are further oxidized by a membrane-bound alcohol dehydrogenase in n-alkanals. ...
CaiA RNA motif
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 ...
Glycolysis
The cytosolic acetyl-CoA can be carboxylated by acetyl-CoA carboxylase into malonyl CoA, the first committed step in the ... Glyceraldehyde phosphate dehydrogenase (GAPDH). an oxidoreductase d-1,3-Bisphosphoglycerate (1,3BPG) ... or it can be combined with acetoacetyl-CoA to form 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) which is the rate limiting step ... The resulting acetyl-CoA enters the citric acid cycle (or Krebs Cycle), where the acetyl group of the acetyl-CoA is converted ...
Choline
... receives 2 acyl groups from acyl-CoA forming a phosphatidic acid. It reacts with cytidine triphosphate to form cytidine ... This is oxidized by mitochondrial or cytosolic betaine-aldehyde dehydrogenases to trimethylglycine.[6] Trimethylglycine is a ...
Fatty acid
"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 ...
Koenzim A
"Acyl carrier protein-specific 4'-phosphopantetheinyl transferase activates 10-formyltetrahydrofolate dehydrogenase". J. Biol. ... Acetil-CoA. *Masni acil-CoA (aktiviran iz masnih kiselina; samo CoA estri su supstrati ta značajne reakcije kao što su sinteze ... Elovson J, Vagelos PR (juli 1968). "Acyl carrier protein. X. Acyl carrier protein synthetase". J. Biol. Chem. 243 (13): 3603-11 ... što su acetil-CoA). Kod ljudi, biosinteza CoA biosinteza uključuje cistein, pantotenat i adenozin trifosfat (ATP).[1] ...
Metabolism
Fatty acids are made by fatty acid synthases that polymerize and then reduce acetyl-CoA units. The acyl chains in the fatty ... Hundreds of separate types of dehydrogenases remove electrons from their substrates and reduce NAD+ into NADH. This reduced ... and this breakdown process involves the release of significant amounts of acetyl-CoA, propionyl-CoA, and pyruvate, which can ... The glycerol enters glycolysis and the fatty acids are broken down by beta oxidation to release acetyl-CoA, which then is fed ...
Beta-Hydroxybutyric acid
3-Hydroxyisovaleryl CoA accumulation can inhibit cellular respiration either directly or via effects on the ratios of acyl CoA: ... The concentration of β-hydroxybutyrate in blood plasma is measured through a test that uses β-hydroxybutyrate dehydrogenase, ... This metabolic pathway is as follows: butyrate→butyryl-CoA→crotonyl-CoA→β-hydroxybutyryl-CoA→poly-β-hydroxybutyrate→D-β-(D-β- ... Metabolic impairment diverts methylcrotonyl CoA to 3-hydroxyisovaleryl CoA in a reaction catalyzed by enoyl-CoA hydratase (22, ...
Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
Medium chain acyl dehydrogenase deficiency Reference, Genetics Home. "LCHAD deficiency". Genetics Home Reference. Retrieved ... "Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: Clinical Presentation and Follow-Up of 50 Patients". Pediatrics. 109 (1 ... Mutations in the HADHA gene lead to inadequate levels of an enzyme called long-chain 3-hydroxyacyl-coenzyme A (CoA) ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency is a rare autosomal recessive fatty acid oxidation disorder that ...
Chromosome 16
ACSF3: encoding enzyme Acyl-CoA synthetase family member 3 ACSM2B: encoding enzyme Acyl-coenzyme A synthetase ACSM2B, ... encoding protein Pyruvate dehydrogenase phosphatase regulatory subunit PKDTS: Polycystic kidney disease, infantile severe, with ... mitochondrial ACSM3: encoding enzyme Acyl-coenzyme A synthetase ACSM3, mitochondrial 2 ADHD1: Attention deficit-hyperactivity ...
HADHB
Middleton B (1994). "The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase ... which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons shorter. The encoded protein can also bind ... Trifunctional protein deficiency is characterized by decreased activity of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), ... Trifunctional enzyme subunit beta, mitochondrial (TP-beta) also known as 3-ketoacyl-CoA thiolase, acetyl-CoA acyltransferase, ...
MCAD
... a gene Medium-chain acyl-CoA dehydrogenase, an enzyme used in lipid metabolism Medium-chain acyl-coenzyme A dehydrogenase ...
Alcanivorax borkumensis
By disrupting an acyl-coenzyme A (CoA) thioesterase gene, Sabirova and colleagues were able to mutate the organism to hyper- ... This sequential pathway first produces alcohols, then alcohol and aldehyde dehydrogenases, and ultimately aldehydes and fatty ...
Short-chain acyl-coenzyme A dehydrogenase deficiency
"Follow-up of patients with short-chain acyl-CoA dehydrogenase and isobutyryl-CoA dehydrogenase deficiencies identified through ... "Orphanet: Short chain acyl CoA dehydrogenase deficiency". www.orpha.net. Retrieved 2016-10-30. Online Mendelian Inheritance in ... Mutations in the ACADS gene lead to inadequate levels of short-chain acyl-CoA dehydrogenase, which is important for breaking ... The symptoms of short-chain acyl-CoA dehydrogenase deficiency may be triggered during illnesses such as viral infections. In ...
Genetic disorder
Examples of this type of disorder are albinism, medium-chain acyl-CoA dehydrogenase deficiency, cystic fibrosis, sickle cell ...
Benzene
The catechol is then metabolized to acetyl CoA and succinyl CoA, used by organisms mainly in the citric acid cycle for energy ... The reaction involves the acylation of benzene (or many other aromatic rings) with an acyl chloride using a strong Lewis acid ... These include cytochrome P450 2E1 (CYP2E1), quinine oxidoreductase (NQ01 or DT-diaphorase or NAD(P)H dehydrogenase (quinone 1 ...
Coenzyme A
A molecule of coenzyme A carrying an acyl group is also referred to as acyl-CoA. When it is not attached to an acyl group, it ... Acetyl-CoA is utilised in the post-translational regulation and allosteric regulation of pyruvate dehydrogenase and carboxylase ... Propionyl-CoA Butyryl-CoA Myristoyl-CoA Crotonyl-CoA Acetoacetyl-CoA Coumaroyl-CoA (used in flavonoid and stilbenoid ... The major route of CoA activity loss is likely the air oxidation of CoA to CoA disulfides. CoA mixed disulfides, such as CoA-S- ...
Acute fatty liver of pregnancy
Deficiency of LCHAD (3-hydroxyacyl-CoA dehydrogenase) leads to an accumulation of medium and long chain fatty acids. When this ... "Disorders of mitochondrial fatty acyl-CoA beta-oxidation" (PDF). Journal of Inherited Metabolic Disease. 22 (4): 442-487. doi: ... IJlst L, Oostheim W, Ruiter JP, Wanders RJ (1997). "Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: ... caused by long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. This leads to decreased metabolism of long chain fatty ...
List of OMIM disorder codes
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... ACADSB 3-hydroxyacyl-coa dehydrogenase deficiency; 231530; HADHSC 3-hydroxyisobutryl-CoA hydrolase deficiency; 250620; HIBCH 3- ... DCX Succinic semialdehyde dehydrogenase deficiency; 271980; ALDH5A1 Succinyl-CoA:3-oxoacid CoA transferase deficiency; 245050; ...
Long-chain-alcohol oxidase
It has been found in certain Candida yeast, where it participates in omega oxidation of fatty acids to produce acyl-CoA for ... Yeast have low levels of fatty alcohol dehydrogenase.) The long-chain alcohol is then oxidized by long-chain fatty aldehyde ... This is different from the pathway found in mammalian tissues, which employs long-chain fatty alcohol dehydrogenase or fatty ... dehydrogenase to a carboxylic acid, also producing NADH from NAD+. Fatty acids can be oxidized again to make dicarboxylic ...
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase
An inducible fatty acyl-CoA oxidase, a noninducible fatty acyl-CoA oxidase, and a noninducible trihydroxycoprostanoyl-CoA ... "Further characterization of the peroxisomal 3-hydroxyacyl-CoA dehydrogenases from rat liver. Relationship between the different ... THC-CoA oxidase, THCA-CoA oxidase, 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA oxidase, 3alpha,7alpha,12alpha- ... Schepers L, Van Veldhoven PP, Casteels M, Eyssen HJ, Mannaerts GP (1990). "Presence of three acyl-CoA oxidases in rat liver ...
List of diseases (M)
... the upper lip Mediastinal endodermal sinus tumors Mediastinal syndrome Mediterranean fever Medium-chain Acyl-CoA dehydrogenase ... Multiple p Multiple acyl-CoA deficiency Multiple carboxylase deficiency, biotin responsive Multiple carboxylase deficiency, ... mixed Müllerian tumor Malignant paroxysmal ventricular tachycardia Mallory-Weiss syndrome Malonic aciduria Malonyl-CoA ... disorder Mal de debarquement Malakoplakia Malaria Male pseudohermaphroditism due to 17-beta-hydroxysteroid dehydrogenase ...
Mitochondrial matrix
β-Oxidation uses pyruvate carboxylase, acyl-CoA dehydrogenase, and β-ketothiolase. Amino acid production is facilitated by ... The citric acid cycle involves acyl-CoA, pyruvate, acetyl-CoA, citrate, isocitrate, α-ketoglutarate, succinyl-CoA, fumarate, ... isocitrate dehydrogenase, α-ketoglutarate dehydrogenase, succinyl-CoA synthetase, fumarase, and malate dehydrogenase. The urea ... in the matrix activates pyruvate dehydrogenase, isocitrate dehydrogenase, and α-ketoglutarate dehydrogenase which increases the ...
HSD17B4
... acyl-CoA oxidase (see, e.g., ACOX1, MIM 609751); the 'D-bifunctional enzyme,' with enoyl-CoA hydratase and D-3-hydroxyacyl-CoA ... 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities". The Journal of Biological Chemistry. 265 ... "D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency: a newly identified ... D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein: its expression in the developing human ...
Branched-chain alpha-keto acid dehydrogenase complex
STEP 3: Acyl group transfer to CoA. The proper arrow-pushing mechanism is shown in Figure 5. *NOTE: The reduced lipoyl arm now ... and converting them to α-Methylbutyryl-CoA, Isobutyryl-CoA and Isovaleryl-CoA respectively. In bacteria, this enzyme ... BCKDC is a member of the mitochondrial α-ketoacid dehydrogenase complex family comprising pyruvate dehydrogenase and alpha- ... Other mitochondrial autoantigens include pyruvate dehydrogenase and branched-chain oxoglutarate dehydrogenase, which are ...
Flavin group
... 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 ...
Medium-chain acyl-CoA dehydrogenase deficiency: MedlinePlus Genetics
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 ...
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD) Medication
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD) * Sections Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency ... encoded search term (Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD)) and Medium-Chain Acyl-CoA Dehydrogenase ( ... Medium-chain acyl-CoA dehydrogenase deficiency in children with non- ketotic hypoglycemia and low carnitine levels. Pediatr Res ... Medium-chain acyl-CoA dehydrogenase deficiency in Saudi Arabia: incidence, genotype, and preventive implications. J Inherit ...
Acyl-CoA dehydrogenase - Wikipedia
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 ...
Orphanet: Medium chain acyl CoA dehydrogenase deficiency
SCOPe 2.07: Fold e.6: Acyl-CoA dehydrogenase NM domain-like
Acyl-CoA dehydrogenase NM domain-like. Timeline for Fold e.6: Acyl-CoA dehydrogenase NM domain-like: *Fold e.6: Acyl-CoA ... Fold e.6: Acyl-CoA dehydrogenase NM domain-like appears in SCOPe 2.06. *Fold e.6: Acyl-CoA dehydrogenase NM domain-like appears ... e.6.1: Acyl-CoA dehydrogenase NM domain-like [56645] (3 families) flavoprotein: binds FAD; constituent families differ in the ... Fold e.6: Acyl-CoA dehydrogenase NM domain-like [56644] (1 superfamily). 2 domains: (1) all-alpha: 5 helices; (2) contains an ...
Medium‐chain and long‐chain acyl CoA dehydrogenase deficiency: Clinical, pathologic and ultrastructural differentiation from...
Long‐chain acyl CoA dehydrogenase deficiency is differentiated from medium‐chain acyl CoA dehydrogenase deficiency by younger ... Long‐chain acyl CoA dehydrogenase deficiency is differentiated from medium‐chain acyl CoA dehydrogenase deficiency by younger ... Long‐chain acyl CoA dehydrogenase deficiency is differentiated from medium‐chain acyl CoA dehydrogenase deficiency by younger ... Long‐chain acyl CoA dehydrogenase deficiency is differentiated from medium‐chain acyl CoA dehydrogenase deficiency by younger ...
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD): Background, Pathophysiology, Epidemiology
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD) * Sections Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency ... At least 3 separate acyl-CoA dehydrogenases are known; they are as follows:. *. Long-chain acyl-CoA dehydrogenase (Length of ... encoded search term (Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency (MCADD)) and Medium-Chain Acyl-CoA Dehydrogenase ( ... Medium-chain acyl-CoA dehydrogenase deficiency in children with non- ketotic hypoglycemia and low carnitine levels. Pediatr Res ...
Enantiomer-specific pharmacokinetics of D,L-3-hydroxybutyrate: Implications for the treatment of multiple acyl-CoA...
Implications for the treatment of multiple acyl-CoA dehydrogenase deficiency. / van Rijt, Willemijn J; Van Hove, Johan L K; Vaz ... Implications for the treatment of multiple acyl-CoA dehydrogenase deficiency. Willemijn J van Rijt, Johan L K Van Hove, ... including multiple acyl-CoA dehydrogenase deficiency (MADD; glutaric aciduria type II). We aimed to improve the understanding ... including multiple acyl-CoA dehydrogenase deficiency (MADD; glutaric aciduria type II). We aimed to improve the understanding ...
SHORT CHAIN ACYL-COA DEHYDROGENASE DEFICIENCY (SCAD)
... Knowledgebase of inborn errors of metabolism ... SHORT CHAIN ACYL-COA DEHYDROGENASE DEFICIENCY (SCAD). Disease. SHORT CHAIN ACYL-COA DEHYDROGENASE DEFICIENCY (SCAD) ... Acyl-CoA dehydrogenase dec (fibroblasts). Acylcarnitine/carnitine ratio inc (urine). Adipic acid inc (urine). Ammonia normal/ ...
Short-Chain Acyl-CoA Dehydrogenase Deficiency - StoryMD
Good Laboratory Practices for Biochemical Genetic Testing and Newborn Screening for Inherited Metabolic Disorders
... a secondary marker for medium chain acyl-CoA dehydrogenase deficiency), immunoreactive trypsinogen (a primary marker for cystic ... Medium-chain acyl-CoA dehydrogenase deficiency. Very long-chain acyl-CoA dehydrogenase deficiency ... Short-chain acyl-CoA dehydrogenase deficiency. Medium/short-chain L-3-hydroxyacyl-CoA ...
Very long chain acyl-CoA dehydrogenase deficiency (VLCADD) - tellmeGen™
Very long-chain acyl CoA dehydrogenase deficiency is an inherited disorder of mitochondrial oxidation of long-chain fatty acids ... Very long chain acyl-CoA dehydrogenase deficiency (VLCADD) Very long chain acyl CoA dehydrogenase deficiency (VLCADD) is an ... Very long chain acyl-CoA dehydrogenase deficiency (VLCADD) ...
Short-chain acyl-CoA dehydrogenase deficiency - Rare Immunology News
... ghr.nlm.nih.gov/condition/short-chain-acyl-coa-dehydrogenase-deficiency. *Jerry Vockley, MD, PhD. Short-Chain Acyl CoA ... called short-chain acyl-CoA dehydrogenase. This enzyme is important for the breakdown of a particular type of fat called short- ... Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is inherited. in an autosomal recessive. pattern, which means both copies ... Short-Chain Acyl-CoA Dehydrogenase Deficiency: Studies in a Large Family Adding to the Complexity of the Disorder. Pediatrics, ...
Incidental Germline Findings for Guiding Cancer Therapy
Medium-chain acyl-CoA dehydrogenase deficiency. Autosomal recessive. No. 23 (2.2). TNFRSF13B Common variable immune deficiency ... short-chain acyl-CoA dehydrogenase; TAR, thrombocytopenia with absent radius. ... Abbreviations: ACMG, American College of Medical Genetics and Genomics; CoA, coenzyme A; P/LP, pathogenic or likely pathogenic ...
Newborn Screening Laboratory Bulletin
Medium chain acyl-CoA dehydrogenase deficiency - Genetic Lifehacks
G/G: medium-chain acyl-coA dehydrogenase deficiency[ref]. *A/G: carrier of one copy of a medium-chain acyl-coA dehydrogenase ... C/C: medium chain acyl-coA dehydrogenase deficiency[ref]. *C/T: carrier of one copy of a medium-chain acyl-coA dehydrogenase ... T/T: medium chain acyl-coA dehydrogenase deficiency[ref]. *C/T: carrier of one copy of a medium-chain acyl-coA dehydrogenase ... T/T: medium-chain acyl-coA dehydrogenase deficiency[ref]. *C/T: carrier of one copy of a medium-chain acyl-coA dehydrogenase ...
MEDIUM CHAIN ACYL-COA DEHYDROGENASE DEFICIENCY (MCAD) , SEQUENCING ACADM GENE - RefLab Genetics
Very long chain acyl-CoA dehydrogenase deficiency - Mountain States GeneticsMountain States Genetics
Natural cure for Acyl-CoA dehydrogenase, medium chain, deficiency of and alternative treatments
Get natural cures for Acyl-CoA dehydrogenase, medium chain, deficiency of that can make a difference in your life or the life ... Acyl-CoA dehydrogenase, medium chain, deficiency of in Texas. Acyl-CoA dehydrogenase, medium chain, deficiency of in Utah. Acyl ... Acyl-CoA dehydrogenase, medium chain, deficiency of by state. Acyl-CoA dehydrogenase, medium chain, deficiency of in Alabama. ... Acyl-CoA dehydrogenase, medium chain, deficiency of in Kansas. Acyl-CoA dehydrogenase, medium chain, deficiency of in Kentucky ...
Overview: What is medium-chain acyl-CoA dehydrogenase (MCAD) deficiency? | ThinkGenetic
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a genetic condition that prevents a persons body from being able to ... How common is medium-chain acyl-CoA dehydrogenase deficiency (MCAD)?. Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is ... ghr.nlm.nih.gov/condition/medium-chain-acyl-coa-dehydrogenase-deficiency *Roe CR. MCAD: Medium Chain acyl CoA Dehydrogenase - ... ghr.nlm.nih.gov/condition/medium-chain-acyl-coa-dehydrogenase-deficiency. *Acyl-CoA Dehydrogenase, medium-chain; ACADM. Online ...
Medium Chain Acyl-CoA Dehydrogenase (ACADM) 2 Mutations | ARUP Laboratories Test Directory
Preferred initial molecular test to confirm a diagnosis or identify carriers of medium chain acyl-CoA dehydrogenase (MCAD) ... Preferred initial molecular test to confirm a diagnosis or identify carriers of medium chain acyl-CoA dehydrogenase (MCAD) ... Background Information for Medium Chain Acyl-CoA Dehydrogenase (ACADM) 2 Mutations:. Characteristics: Limited mitochondrial ...
Welcome to PhenoDis
Acyl-CoA dehydrogenase 9 deficiency General Information (adopted from Orphanet):. Synonyms, Signs: ACAD9 deficiency. ... Acyl-CoA dehydrogenase 9 (ACAD9) is an assembly factor for mitochondrial respiratory chain Complex I (CI). The tissue ... 2007) that ACAD9 and very long-chain acyl-CoA dehydrogenase (ACADVL; 609575) are unable to compensate for each other in ...
Production and release of acylcarnitines by primary myotubes reflect the differences in fasting fat oxidation of the donors
Part II: PUBLIC HEALTH ASSESSMENT Chapter 7 | CDC
Medium chain acyl-coA dehydrogenase deficiency (MCADD). HuGE Reviews 1999. *PrevChapter 1 ... For instance, what data are needed before a new genetic test (e.g., for MCADD - medium-chain acyl dehydrogenase deficiency) is ... Medium-chain acyl dehydrogenase deficiency (MCADD), a recently identified disorder of fatty acid metabolism, has been ...
Metabolic disorder in infants: Symptoms, treatments, and outlook
Medium-chain acyl-CoA dehydrogenase deficiency. (2015). https://medlineplus.gov/genetics/condition/medium-chain-acyl-coa- ... Babies with medium-chain acyl-CoA dehydrogenase deficiency cannot convert certain fats to energy. As a result, they may require ... dehydrogenase-deficiency/. *. Newborn screening process. (2021). https://newbornscreening.hrsa.gov/newborn-screening-process# ...
MSMEG 3392 MSMEG 3392 Acyl-CoA dehydrogenase (EC 1.3.8.-) (Acyl-CoA dehydrogenase domain protein)Mycobacterium smegmatis ...
Immunochemical characterization of variant Medium-chain acyl-CoA dehydrogenase in fibroblasts from patients with Medium-chain...
Newborn Metabolic Screenings in PA, NJ and DE | Children's Hospital of Philadelphia
Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD). fatty acid oxidation. yes. yes. yes. ... Medium-chain acyl-CoA dehydrogenase deficiency (MCAD). fatty acid oxidation. yes. yes. yes. ... Short-chain acyl-CoA dehydrogenase deficiency. fatty acid oxidation. no. yes. yes. ... Medium/short-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency (M/SCHAD). fatty acid oxidation. no. yes. no. ...
Table 1 - Community Outbreak of Adenovirus, Taiwan, 2011 - Volume 18, Number 11-November 2012 - Emerging Infectious Diseases...
DeficiencyMultiple acyl-CoA dehydrogeMCADACADMEnzymeCarnitinePyruvateFlavoproteinProteinChainAcetyl-CoAFatty acylSynthetaseSubstrateAciduriaTransferring the electronsMitochondriaG6PDDiseasePutativeGlutamateLong-chain acyl-CoMitochondrialEnoyl-CoA hydraCoenzymeNADHAldehyde dehydrogenaseGenesIsobutyryl-CoA dehydrogenaseACADSElectron-transferSuccinatePeroxisomalCitric acidProteinsEnzymesNADPAcetylOxidoreductasesLactateReactionPMID
Deficiency55
- 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)
- Medium-chain acyl-CoA dehydrogenase deficiency in children with non- ketotic hypoglycemia and low carnitine levels. (medscape.com)
- Population spectrum of ACADM genotypes correlated to biochemical phenotypes in newborn screening for medium-chain acyl-CoA dehydrogenase deficiency. (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)
- Risk stratification by residual enzyme activity after newborn screening for medium-chain acyl-CoA dehyrogenase deficiency: data from a cohort study. (medscape.com)
- Abnormal screening in a healthy infant of a mother with undiagnosed medium-chain acyl-coA dehydrogenase deficiency. (medscape.com)
- Newborn screening for medium-chain acyl-CoA dehydrogenase deficiency: regional experience and high incidence of carnitine deficiency. (medscape.com)
- Medium-chain acyl-CoA dehydrogenase deficiency: genotype-biochemical phenotype correlations. (medscape.com)
- Medium chain acyl-CoA dehydrogenase (MCAD) deficiency (MCADD) is an inborn error of mitochondrial fatty acid oxidation characterized by a rapidly progressive metabolic crisis, often presenting as hypoketotic hypoglycemia, lethargy, vomiting, seizures and coma, which can be fatal in the absence of emergency medical intervention. (orpha.net)
- Differential diagnosis includes other disorders of mitochondrial fatty acid oxidation including multiple acyl-CoA dehydrogenase deficiency (MADD) (see this term). (orpha.net)
- The clinical and pathologic findings in 12 patients with medium‐chain acyl CoA dehydrogenase deficiency and three patients with long‐chain acyl CoA dehydrogenase deficiency are summarized. (elsevier.com)
- Younger age at presentation, history of unexplained sibling death, a previous episode of lethargy, hypoglycemia or acidosis precipitated by fasting stress and only mildly elevated serum transaminases with normal or only mildly prolonged prothrombin time may all suggest an acyl CoA dehydrogenase deficiency. (elsevier.com)
- Long‐chain acyl CoA dehydrogenase deficiency is differentiated from medium‐chain acyl CoA dehydrogenase deficiency by younger age at presentation, more profound cardiorespiratory depression, evidence of cardiomyopathy, and sequelae of muscle weakness, hypotonia and developmental delay. (elsevier.com)
- Four of seven cases of acyl CoA dehydrogenase deficiency showed some variations from normal in the appearance of the hepatocyte mitochondria. (elsevier.com)
- In 1983, Gregersen et al demonstrated a medium-chain acyl-coenzyme A (CoA) dehydrogenase (MCAD) deficiency in a patient with hypoketotic hypoglycemia. (medscape.com)
- Very long chain acyl CoA dehydrogenase deficiency (VLCADD) is an inherited disorder of the mitochondrial oxidation of long chain fatty acids with variable phenotypes which include: cardiomyopathy, hypocetotic hypoglycemia, liver disease, exercise intolerance and rhabdomyolysis. (tellmegen.com)
- Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations . (rareimmunology.com)
- Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is a rare genetic condition that prevents the body from converting certain fats (called short-chain fatty acids) into energy. (rareimmunology.com)
- [1] These mutations lead to a shortage (deficiency) of an enzyme known as short-chain acyl-CoA dehydrogenase, which is involved in the breakdown of short-chain fatty acids. (rareimmunology.com)
- Mediumchain acyl-CoA dehydrogenase deficiency: human genome epidemiology review. (cdc.gov)
- Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is an " inborn error of metabolism" which impairs the body's ability to break down medium-chain fatty acids for fuel. (geneticlifehacks.com)
- The following genetic mutations in the ACADM gene are linked with medium-chain acyl-CoA deficiency. (geneticlifehacks.com)
- Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD) is a rare hereditary disease that is the result of the lack of an enzyme required to convert fat to energy. (naturalcurefor.com)
- Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a genetic condition that prevents a person's body from being able to break down a specific type of body fat to make energy. (thinkgenetic.com)
- Are there other names for medium-chain acyl-CoA dehydrogenase deficiency (MCAD)? (thinkgenetic.com)
- Preferred initial 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)
- People with medium chain acyl-CoA dehydrogenase deficiency (MCADD) cannot burn fat for energy. (cdc.gov)
- Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (EC 1.3.99.10) resulting in the accumulation of derivatives of isovaleryl-CoA. (hmdb.ca)
- 16602101 ). Moreover, isovalerylcarnitine is found to be associated with celiac disease and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD), which are also inborn errors of metabolism. (hmdb.ca)
- Peripheral parenteral nutrition formula with medium-chain acyl-coa dehydrogenase deficiency. (aaan.org)
- Ryder was born with VLCAD , which stands for Very-long-chain acyl-CoA dehydrogenase deficiency. (monstersandcritics.com)
- Mutations in ACADS have been associated with Short Chain Acyl-CoA Dehydrogenase Deficiency. (prospecbio.com)
- Anderson DR, Viau K, Botto LD, Pasquali M, Longo N . Clinical and biochemical outcomes of patients with medium-chain acyl-CoA dehydrogenase deficiency. (aruplab.com)
- Ryder is diagnosed with VLCAD ("very long-chain acyl-CoA dehydrogenase deficiency") which means "she can't process fatty foods into energy," Wharton explained to Us in December 2017. (usmagazine.com)
- The lab is currently developing an AVV gene therapy approach to treat a unique chorioretinipathy observed in patient and our mouse model of Long-chain 3-hydroxy Acyl-CoA dehydrogenase deficiency. (ohsu.edu)
- Background: We describe early health services utilization for children diagnosed with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency through newborn screening in Ontario, Canada, relative to a screen negative comparison cohort. (uwo.ca)
- Alatibi KI , Wehbe Z , Spiekerkoetter U , Tucci S . Sex-specific perturbation of complex lipids in response to medium-chain fatty acids in very long-chain acyl-CoA dehydrogenase deficiency. (wjgnet.com)
- Examples of some of the disorders that are included in the recommended panel are medium-chain acyl-CoA dehydrogenase deficiency (MCADD), cystic fibrosis (CF), congenital hypothyroidism (CH), congenital adrenal hyperplasia (CAH), and congenital deafness (HEAR). (savebabies.org)
- Chapter waddell medium-chain acyl-coa dehydrogenase scad deficiency is a fluid-filled space between the right upper quadrant pain also explains how gating can occur with myopia and by itself therefore, parents should know their names. (aaan.org)
- BACKGROUND: Several recessive Mendelian disorders are common in Europeans, including cystic fibrosis (CFTR), medium-chain-acyl-Co-A-dehydrogenase deficiency (ACADM), phenylketonuria (PAH) and alpha 1-antitrypsin deficiency (SERPINA1). (ox.ac.uk)
- Background: Medium chain acyl-CoA dehydrogenase deficiency (MCADD) is an autosomal recessive fatty acid β-oxidation defect. (eurospe.org)
- This variant is interpreted as a Uncertain Significance - Conflicting Evidence, for Very long chain acyl-coa dehydrogenase deficiency, in Autosomal Recessive manner. (utah.edu)
- The observed variant frequency within African or African-American control individuals in the gnomAD database is approximately 10 fold of the estimated maximal expected allele frequency for a pathogenic variant in ACADVL causing Very Long Chain Acyl-CoA Dehydrogenase Deficiency phenotype (0.0029), strongly suggesting that the variant is a benign polymorphism found primarily in populations of African or African-American origin. (utah.edu)
- Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is an inherited (genetic) condition that prevents the body from breaking down certain fats and turning them into energy. (hrsa.gov)
- 2021. Multiple acyl-CoA dehydrogenase deficiency kills Mycobacterium tuberculosis in vitro and during infection . (immunopaedia.org.za)
- This nutrition guideline is specific to the very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) form of LC-FAOD. (faodinfocushcp.com)
- min stuff, not recurrent Arbeits-, situation view Chain Acyl-CoA Dehydrogenase Deficiency( SCADD) is a stage in which the project is to test life-threatening dangers because an conjuctiva means sometimes ankylosing or no manipulating right. (lakesinclair.org)
- Prevention phase on initial presentation of medium-chain acyl-coa dehydrogenase deficiency is the preferable posture for rest, since lying down increases blood return will occur if hypercapnia is of two long heavy chains called the alveolar pressure and low cholesterol foods with limited exceptions, most pharmacologic agents are also dose-dependent. (albionfoundation.org)
Multiple acyl-CoA dehydroge1
- Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. (biossusa.com)
MCAD5
- 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)
- 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)
- MCAD: Medium Chain acyl CoA Dehydrogenase - Information for Families. (thinkgenetic.com)
- The decreased expression of medium-chain acyl-coenzyme A dehydrogenase (MCAD) in these myotubes can explain the higher rate of incomplete fatty acid oxidation. (nih.gov)
- The ACADM gene contains instructions for making an enzyme called medium-chain acyl-CoA dehydrogenase (MCAD). (23andme.com)
ACADM1
- Fatty acyl coenzyme A dehydrogenase (ambiguous), Acyl coenzyme A dehydrogenase (ambiguous), Acyl dehydrogenase (ambiguous), Fatty-acyl-CoA dehydrogenase (ambiguous), Acyl CoA dehydrogenase (ambiguous), General acyl CoA dehydrogenase (ambiguous), Medium-chain acyl-coenzyme A dehydrogenase, Acyl-CoA:(acceptor) 2,3-oxidoreductase (ambiguous), ACADM (gene name). (umassmed.edu)
Enzyme8
- 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)
- 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)
- Entry into the beta-oxidation cycle requires the action of acyl-CoA dehydrogenase, the first enzyme in the sequence, which removes electrons from the alpha-carbon and the beta-carbon, introducing a double bond. (medscape.com)
- The enzyme is most active toward long-chain acyl-CoAs such as C 14 , C 16 and C 18 , but is also active with very-long-chain acyl-CoAs up to 24 carbons. (enzyme-database.org)
- Vertebrate Acyl CoA synthetase family member 4 (ACSF4-U26) is a β-alanine-activating enzyme homologous to bacterial non-ribosomal peptide synthetase. (nih.gov)
- This is accomplished through the activation of three thermogenic enzymes: Glycerol-3-Phosphate Dehydrogenase, Malic Enzyme and Fatty Acyl CoA Oxidase. (vitanatural.net)
- The enzyme is important in the breakdown of medium chain fats into acetyl-CoA to produce ketones, alternative energy source when glucose hepatic glycogen stores become depleted during prolonged fasting. (eurospe.org)
Carnitine3
- This acyl-CoA is linked to carnitine by the action of CPT I, with simultaneous transport across the mitochondrial membrane barrier. (medscape.com)
- Once inside the mitochondrion, the action of CPT II at the inner surface of the membrane releases free carnitine, which exits to the cytosol and leaves behind the acyl-CoA molecule. (medscape.com)
- Carnitine binds acyl residues and helps in their elimination, decreasing the number of acyl residues conjugated with coenzyme A (CoA) and increasing the ratio between free and acylated CoA. (medscape.com)
Pyruvate2
- RELMα partially increases glycolytic oxidation and increases pyruvate dehydrogenase kinase (PDK-1, 4) gene expression which also inhibits conversion of pyruvate to acetyl-CoA for TCA cycle. (frontiersin.org)
- Also, decarboxylation of pyruvate and α-ketoglutarate and fatty acyl-CoA dehydrogenase (in fatty acid oxidation) also requires FAD. (skinactives.com)
Flavoprotein3
- ACADS is a tetrameric mitochondrial flavoprotein, which is part of the acyl-CoA dehydrogenase family. (prospecbio.com)
- As an example of the use of this method, we determine the reduction potential of the covalently bound FAD cofactor ( E m = -55 mV) in the SdhA flavoprotein subunit of succinate dehydrogenase from Escherichia coli . (bio-protocol.org)
- Here we show an example of how this method is applied to determine the reduction potential of covalently bound FAD in the SdhA flavoprotein of succinate dehydrogenase. (bio-protocol.org)
Protein1
- 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)
Chain15
- Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism. (medscape.com)
- 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)
- 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 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)
- In the cytosol, a saturated, straight-chain fatty acid molecule with no double bonds is activated by the action of fatty acyl-CoA synthetase to form its corresponding acyl-CoA. (medscape.com)
- Acyl-CoA dehydrogenase 9 (ACAD9) is an assembly factor for mitochondrial respiratory chain Complex I (CI). (helmholtz-muenchen.de)
- The C2 overall structure resembles the folding of medium-chain acyl-CoA dehydrogenase. (proteopedia.org)
- Etomoxir (inhibitor of long chain FA) also limits FA availability to mitochondria, thus, less FAO (β-oxidation) further limits Acetyl-CoA production (substrate for TCA cycle). (frontiersin.org)
- a very-long-chain acyl-CoA = an acyl-CoA thioester where the acyl chain contains 23 or more carbon atoms. (enzyme-database.org)
- Its specific activity towards palmitoyl-CoA is more than 10-fold that of the long-chain acyl-CoA dehydrogenase [1]. (enzyme-database.org)
- EC 1.3.8.1 , short-chain acyl-CoA dehydrogenase, EC 1.3.8.7 , medium-chain acyl-CoA dehydrogenase, and EC 1.3.8.8 , long-chain acyl-CoA dehydrogenase. (enzyme-database.org)
- I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase. (enzyme-database.org)
- Health services use among children diagnosed with medium-chain acyl-Co" by Maria D. Karaceper, Sara D. Khangura et al. (uwo.ca)
- In the bryozoan Bugula neritina, butenolide bound to very long chain acyl-CoA dehydrogenase (ACADVL), actin, and glutathione S-transferases (GSTs). (edu.sa)
Acetyl-CoA3
- 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)
- Cleavage of the 3-keto compound at the now unstable alpha-beta carbon bond and transfer of another CoA moiety to the new fragment results in 2 products: acetyl-CoA, composed of the carbonyl and original alpha-carbon from the starting molecule, and a new fatty acyl-CoA that is 2 carbons shorter than the original molecule. (medscape.com)
- In the barnacle Balanus (=Amphibalanus) amphitrite, butenolide bound to acetyl-CoA acetyltransferase 1 (ACAT1), which is involved in ketone body metabolism. (edu.sa)
Fatty acyl1
- and (v) peroxisomal downregulation, as demonstrated by levels and distribution of fatty acyl β -oxidation enzymes. (hindawi.com)
Synthetase3
- Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome. (nih.gov)
- acyl-CoA synthetase long c. (gsea-msigdb.org)
- UST020129-010, butenolide bound to succinyl-CoA synthetase β subunit (SCSβ) and inhibited bacterial growth. (edu.sa)
Substrate2
- 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)
- There are a total of 4 active sites within the tetramer, each of which contains a single FAD molecule and an acyl-CoA substrate binding site. (wikipedia.org)
Aciduria1
- C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects. (medscape.com)
Transferring the electrons1
- Electron transfer flavoproteins (ETFs) serve as specific electron acceptors for primary dehydrogenases, transferring the electrons to terminal respiratory systems. (embl.de)
Mitochondria2
- 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)
- Regulation of the intramitochondrial free CoA also is affected, with accumulation of acyl-CoA esters in the mitochondria. (medscape.com)
G6PD1
- Calvaresi EC, Genzen JR . Evaluating Percentage-Based Reporting of Glucose-6-Phosphate Dehydrogenase (G6PD) Enzymatic Activity. (aruplab.com)
Disease1
- Au cours des trois premiers mois depuis son identification initiale en décembre 2019, le SARS-CoV-2 a causé plus de 800 000 cas confirmés et plus de 40 000 décès dans le monde du syndrome respiratoire aigu sévère associé COVID-19 (coronavirus disease 2019) 1 . (anticorps-enligne.fr)
Putative2
- Cloning and characterization of a novel human homolog* of mouse U26, a putative PQQ-dependent AAS dehydrogenase. (nih.gov)
- Genes identified in this manner encoded three putative (methyl)malonate semialdehyde dehydrogenases ( mmsA1, mmsA2 and mmsA3 ) and two putative dehydrogenases ( hpdH and hbdH ). (biomedcentral.com)
Glutamate1
- glutamate dehydrogenase mutations are responsible for hyperammonemia-hyperinsulinism (brief summary: Hussain et al. (uchicago.edu)
Long-chain acyl-Co2
- This gene provides instructions for making an enzyme called very long-chain acyl-CoA dehydrogenase, which is required to break down (metabolize) a group of fats called very long-chain fatty acids. (medlineplus.gov)
- Located on chromosome 17, the ACADVL gene encodes the very long-chain acyl-CoA dehydrogenase protein (VLCAD), an enzyme required to break down (metabolize) a group of fats called very long-chain fatty acids. (snpedia.com)
Mitochondrial3
- Both mitochondrial acyl-CoA dehydrogenases (ACAD) and peroxisomal acyl-CoA oxidases (AXO) catalyze the alpha,beta dehydrogenation of the corresponding trans-enoyl-CoA by FAD, which becomes reduced. (umbc.edu)
- This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. (empiregenomics.com)
- Background: Mitochondrial acyl-CoA dehydrogenase family member 9 (ACAD9) is essential for the assembly of mitochondrial respiratory chain complex I. Disease causing biallelic variants in ACAD9 have been reported in individuals presenting with lactic acidosis and cardiomyopathy. (uni-frankfurt.de)
Enoyl-CoA hydra3
- enoyl-CoA hydratase domain containin. (gsea-msigdb.org)
- 3R)-3-hydroxyacyl-CoA dehydrogenase / enoyl-CoA hydratase 2 [EC:1.1.1. (kegg.jp)
- The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. (nih.gov)
Coenzyme3
- acyl coenzyme A dehydrogenase. (expasy.org)
- This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. (gen-script.com)
- These are acyl CoAs where the group acylated to the coenzyme A moiety is a long aliphatic chain of 13 to 21 carbon atoms. (ymdb.ca)
NADH2
- However their most well know role is in energy metabolism pathways where FMN is required for the activity of NADH dehydrogenase (ubiquinone) in the electron transport chain, and FAD is required for the enzymes succinate dehydrogenase (citric acid cycle) and fatty acyl CoA dehydrogenase (β-oxidation of fatty acid). (robertbarrington.net)
- There was one change made in the network provided by CellNetAnalyzer for the NADH dehydrogenase reaction, annotated as NADHdehydro in CellNetAnalyzer. (igem.org)
Aldehyde dehydrogenase3
- aldehyde dehydrogenase B [Ensembl]. (ntu.edu.sg)
- putative NAD-dependent aldehyde dehydrogenase [Ensembl]. (ntu.edu.sg)
- The artemisinin content was analysed and compared between transformed and untransformed plants with the help of LC-MS/MS. Expression of key genes [Cytochrome P450 ( CYP71AV1 ), aldehyde dehydrogenase 1 ( ALDH1 ), amorpha-4, 11 diene synthase ( ADS )] in the biosynthetic pathway of artemisinin and gene for trichome development and sesquiterpenoid biosynthetic ( TFAR1 ) were measured using Quantitative real time PCR (qRT-PCR). (biomedcentral.com)
Genes1
- One month post-MI, the expression of several metabolic genes, i.e., acyl-CoA synthetase (− 50%), muscle-type carnitine palmitoyl transferase 1 (− 37%) and citrate synthase (− 28%), was significantly reduced in the surviving myocardium. (mmu.ac.uk)
Isobutyryl-CoA dehydrogenase1
- IBD stands for "isobutyryl-CoA dehydrogenase. (newbornscreening.info)
ACADS2
- Landry and colleagues noted that for decades, ACADS was known to contain CoA with an extra sulfur bound to it, called CoA persulfide. (news-medical.net)
- When CoA persulfide is bound to ACADS, it essentially blocks its function, says Landry, preventing the utilization of butyrate. (news-medical.net)
Electron-transfer1
- Flavoprotein dehydrogenases oxidize organic substrates and mainly use quinones and electron transfer proteins as electron acceptors. (schoolbag.info)
Succinate3
- NADP-dependent succinate-semialdehyde dehydrogenase I. (ntu.edu.sg)
- succinate-semialdehyde dehydrogenase I [Ensembl]. (ntu.edu.sg)
- Succinate-semialdehyde dehydrogenase [NADP(+)] GabD. (ntu.edu.sg)
Peroxisomal1
- encodes an peroxisomal NAD-malate dehydrogenase that is involved in fatty acid beta-oxidation through providing NAD to the process of converting fatty acyl CoA to acetyl CoA. (edu.au)
Citric acid1
- These ketone bodies are readily picked up by the extra-hepatic tissues, and converted into acetyl-CoA which then enters the citric acid cycle and is oxidized in the mitochondria for energy. (diabetestalk.net)
Proteins2
- This classification also contains few proteins with not known function and they are classified into some families only by some relations between primary structures with other well known dehydrogenases. (dehydrogenases.com)
- However, at high initial glycerol concentrations, some of the proteins involved in the butyrate synthesis pathways such as a putative ethanol dehydrogenase (CBY_3753) and a 3-hydroxybutyryl-CoA dehydrogenase (CBY_3045) were up-regulated in both exponential and stationary growth phases. (springeropen.com)
Enzymes1
- A subclass of enzymes which includes all dehydrogenases acting on carbon-carbon bonds. (nih.gov)
NADP2
- Furthermore, to optimize the redox imbalance in production of fatty acids from glucose, two endogenous NAD + -dependent glycerol-3-phosphate dehydrogenases were deleted, and a heterologous NADP + -dependent glyceraldehyde-3-phosphate dehydrogenase was introduced. (researchsquare.com)
- 3gdf (Zn: 4) - Crystal Structure of the Nadp-Dependent Mannitol Dehydrogenase From Cladosporium Herbarum. (atomistry.com)
Acetyl6
- The data show that, although the acetyl moiety of acetyl-CoA generated in brain mitochondria is largely translocated as citrate from these organelles to the cytosol, a cytosolic pathway exists by which acetoacetate is converted directly into acetyl-COA in this cellular compartment. (diabetestalk.net)
- When two acetyl-CoA molecules lose their -CoAs, (or Co-enzyme A groups) they can form a (covalent) dimer called acetoacetate. (diabetestalk.net)
- Both are 4-carbon molecules, that can readily be converted back into acetyl-CoA by most tissues of the body, with the notable exception of the liver. (diabetestalk.net)
- A bacterial pyruvate dehydrogenase (PDH) complex expressed in the yeast cytosol was reported to enable production of cytosolic acetyl-CoA with lower energy cost and no toxic intermediate. (researchsquare.com)
- We demonstrated that cytosolic PDH pathway enabled more efficient acetyl-CoA provision with the lower ATP cost, and improved FFA production. (researchsquare.com)
- Together with engineering of the redox factor rebalance, the cytosolic PDH pathway could achieve high level of FFA production at similar levels of other best acetyl-CoA producing pathways. (researchsquare.com)
Oxidoreductases1
- This classification of dehydrogenases and oxidoreductases is based mainly on the primary sequences. (dehydrogenases.com)
Lactate1
- Lactate dehydrogenase (EC 1.1.2.3) [ ( PUBMED:3004948 ) ], an enzyme that consists of a flavodehydrogenase domain and a heme-binding domain called cytochrome b2. (embl.de)
Reaction2
- the polymerization reaction just consumes (R)-3-hydroxybutanoyl-CoA. (igem.org)
- 3giq (Zn: 4) - Crystal Structure of N-Acyl-D-Glutamate Deacylase From Bordetella Bronchiseptica Complexed with Zinc and Phosphonate Inhibitor, A Mimic of the Reaction Tetrahedral Intermediate. (atomistry.com)
PMID1
- http://dx.doi.org/10.1210/en.136.10.4640 PMid:7664684 Han ZB, Lan GC, Wu YG, Han D, et al. (geneticsmr.com)