CoenzymeDeficiencyMedium-chain acyl-CoA dehydroge2023Reductase inhibitorsEnzymeDehydrogenaseRiboflavin responsiveMitochondrialTandem mass spectroLigaseEstersSubunitMoleculeCOMPOUNDMCADSynthaseInhibitionReactionMetabolismMoietyChairpersonGene2020PathwayDefectsCommission on AuEnzymesUtilizationMyopathyDefectProteinCompoundsCarbonClinical2002Homo2001SpeciesAcidAsymptomaticViewActivity
Coenzyme9
- 3-Hydroxybenzoyl-CoA is a molecule formed by condensing the thiol group of coenzyme A (CoA) with the carboxylic acid group of 3-hydroxybenzoic acid. (wikipedia.org)
- An oxo-fatty acyl-CoA that results from the formal condensation of the thiol group of coenzyme A with the carboxylic acid group of 3-oxolauroic acid. (ebi.ac.uk)
- Statins act by inhibiting 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase and thereby reducing cholesterol synthesis. (nih.gov)
- The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase (LCKT). (medscape.com)
- The reductive acetyl-coenzyme A (acetyl-CoA) pathway, also known as the Wood-Ljungdahl pathway, allows reduction and condensation of two molecules of carbon dioxide (CO 2 ) to build the acetyl-group of acetyl-CoA. (degruyter.com)
- These are organic compounds containing a coenzyme A derivative, which is 3-oxo acylated long aliphatic chain of 13 to 21 carbon atoms. (ymdb.ca)
- In 1961, Childs et al published the earliest clinical report of a patient who was ultimately found to be affected by a deficiency of propionyl coenzyme A (CoA) carboxylase (ie, propionic acidemia). (medscape.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)
- 3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors (statins) effectively reduce cholesterol levels and decrease the incidence of cardiovascular and cerebrovascular events (4). (cdc.gov)
Deficiency31
- 3-methylglutaconyl-CoA hydratase deficiency is an inherited condition that causes neurological problems. (medlineplus.gov)
- In some cases, signs and symptoms of 3-methylglutaconyl-CoA hydratase deficiency begin in adulthood, often in a person's twenties or thirties. (medlineplus.gov)
- Affected individuals who show symptoms of 3-methylglutaconyl-CoA hydratase deficiency in childhood often go on to develop leukoencephalopathy and other neurological problems in adulthood. (medlineplus.gov)
- All people with 3-methylglutaconyl-CoA hydratase deficiency accumulate large amounts of a substance called 3-methylglutaconic acid in their body fluids. (medlineplus.gov)
- 3-methylglutaconyl-CoA hydratase deficiency is one of a group of metabolic disorders that can be diagnosed by the presence of increased levels 3-methylglutaconic acid in urine (3-methylglutaconic aciduria). (medlineplus.gov)
- People with 3-methylglutaconyl-CoA hydratase deficiency also have high urine levels of another acid called 3-methylglutaric acid. (medlineplus.gov)
- Mutations in the AUH gene cause 3-methylglutaconyl-CoA hydratase deficiency. (medlineplus.gov)
- Researchers speculate that an accumulation of these acids in the fluid that surrounds and protects the brain and spinal cord (the cerebrospinal fluid or CSF) can damage these structures and contribute to the neurological features of 3-methylglutaconyl-CoA hydratase deficiency. (medlineplus.gov)
- Because the age at which the condition begins varies widely and because the signs and symptoms improve in some affected children, researchers speculate that other genes or environmental factors may play a role in the features of 3-methylglutaconyl-CoA hydratase deficiency. (medlineplus.gov)
- Some patients who are deficient in all 3 enzymatic activities of the protein have been described, although most have an isolated LCHAD deficiency, which results in the inability to metabolize long-chain fatty acids. (medscape.com)
- Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency (LCHAD) deficiency. (medscape.com)
- Patients with LCHAD deficiency may develop a profound CNS deficiency of docosahexanoic acid ethyl ester (DHA), 22:6n-3. (medscape.com)
- The etiology of the severe peripheral neuropathy of trifunctional protein deficiency may result from the unique metabolite, 3-keto-acyl-CoA, after conversion to a methylketone via spontaneous decarboxylation. (medscape.com)
- 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)
- Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency. (harvard.edu)
- Coordinated and reversible reduction of enzymes involved in terminal oxidative metabolism in skeletal muscle mitochondria from a riboflavin-responsive, multiple acyl-CoA dehydrogenase deficiency patient. (harvard.edu)
- Anesthetic considerations for a patient with compound heterozygous medium-chain Acyl-CoA dehydrogenase deficiency. (harvard.edu)
- Neonatal screening for medium--chain acyl-CoA dehydrogenase deficiency. (harvard.edu)
- Compound heterozygosity in four asymptomatic siblings with medium-chain acyl-CoA dehydrogenase deficiency. (harvard.edu)
- Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy. (harvard.edu)
- Subsequent work led to further delineation of another disorder, initially called multiple carboxylase deficiency, which includes deficiency of propionyl CoA carboxylase activity in addition to defects in other carboxylases. (medscape.com)
- GA-1 is an autosomal recessive disorder caused by deficiency of glutaryl-CoA dehydrogenase, a mitochon- drial enzyme involved in the metabolism of lysine, hydroxylysine and tryptophan. (who.int)
Medium-chain acyl-CoA dehydroge2
- Structure of the transition state analog of medium-chain acyl-CoA dehydrogenase. (rcsb.org)
- The flavoenzyme medium-chain acyl-CoA dehydrogenase (MCAD) eliminates the alpha-proton of the substrate analog, 3-thiaoctanoyl-CoA (3S-C8-CoA), to form a charge-transfer complex with deprotonated 3S-C8-CoA. (rcsb.org)
20231
- In clarifying that the principle of quantum meruit in Torreta should not benefit the petitioner in Topbest Printing Corporation v. COA (G.R. No. 261207, August 22, 2023), the Supreme Court, speaking through Associate Justice Maria Filomena D. Singh, ruled that this principle of equity should not apply in instances where the COA decision sought to be assailed has already attained finality. (gov.ph)
Reductase inhibitors2
- Recent clinical evidence indicates a potential for skeletal muscle toxicity after therapy with HMG-CoA reductase inhibitors (HMGRIs) in man. (aspetjournals.org)
- Pleiotropic effects of HMG-CoA reductase inhibitors. (bvsalud.org)
Enzyme8
- In organisms such as plants, this can be formed using the 3-hydroxybenzoate-CoA ligase enzyme. (wikipedia.org)
- It can be reduced to 3-hydroxycyclohexa-1,5-diene-1-carbonyl-CoA by reduced ferredoxin and adenosine triphosphate using the benzoyl-CoA reductase enzyme. (wikipedia.org)
- A human metabolite involved in the fatty acid elongation in the mitochondrial pathway, its formation from acetyl-CoA is catalysed by the enzyme acetyl-CoA C-acyltransferase. (ebi.ac.uk)
- The deprotonated 3S-C8-CoA was clearly located within the active-site cleft of the enzyme. (rcsb.org)
- Results: In this study the acetoacetyl-CoA reductase gene from C. necator (CnAAR), a NADPH-dependent enzyme, was replaced by the NADH-dependent AAR gene from Allochromatium vinosum (AvAAR) in recombinant xylose-utilizing S. cerevisiae and PHB production was compared. (lu.se)
- The enzyme from Metallosphaera sedula acts nearly equally as well on (S)-3-hydroxybutanoyl-CoA but not (R)-3-hydroxybutanoyl-CoA. (expasy.org)
- We have cloned previously the human long-chain fatty acid-CoA ligase 4 (FACL4), which is a central enzyme in controlling the free arachidonic acid level in cells and thereby regulating eicosanoid production. (nih.gov)
- 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)
Dehydrogenase4
- Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the trifunctional protein of the inner mitochondrial membrane. (medscape.com)
- In addition to the central catalysts, CO dehydrogenase and acetyl-CoA synthase, ATPases are needed in the pathway. (degruyter.com)
- Ni-Zn-[Fe4-S4] and Ni-Ni-[Fe4-S4] clusters in closed and open subunits of acetyl-CoA synthase/carbon monoxide dehydrogenase. (degruyter.com)
- of the head shows fronto-temporal atrophy carnitine levels in urine were elevated and and bilateral subdural haemorrhage glutaryl-CoA dehydrogenase activity in cul- tured fibroblasts was low. (who.int)
Riboflavin responsive1
- C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects. (medscape.com)
Mitochondrial2
- the mitochondrial CoA, thus freed, can participate in other reactions or once again become involved in formation of propionyl CoA. (medscape.com)
- This acyl-CoA is linked to carnitine by the action of CPT I, with simultaneous transport across the mitochondrial membrane barrier. (medscape.com)
Tandem mass spectro1
- Measurement of tissue acyl-CoAs using flow-injection tandem mass spectrometry: acyl-CoA profiles in short-chain fatty acid oxidation defects. (harvard.edu)
Ligase1
- Fatty acid utilization is initiated by fatty acid-CoA ligase, which converts free fatty acids into fatty acyl-CoA esters. (nih.gov)
Esters1
- Thus, the clinical features may result 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. (medscape.com)
Subunit1
- the second locus, on chromosome 3, controls synthesis of the β subunit. (medscape.com)
Molecule6
- Thus, 3-oxopalmitoyl-CoA is considered to be a fatty ester lipid molecule. (ymdb.ca)
- 3-oxopalmitoyl-CoA is a very hydrophobic molecule, practically insoluble (in water), and relatively neutral. (ymdb.ca)
- 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)
- 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)
- The next step is the introduction of a water molecule and resaturation of the double bond to form fatty enoyl-CoA. (medscape.com)
- 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)
COMPOUND1
- Oxidation of the hydroxyl substituent group on the beta-carbon creates an inherently unstable beta-ketoacyl-CoA compound. (medscape.com)
MCAD1
- The crystalline complex was obtained by co-crystallizing MCAD in the oxidized form with 3S-C8-CoA. (rcsb.org)
Synthase1
- The bacterial pathway is comprised of the three enzymes β-ketothiolase, acetoacetyl-CoA reductase (AAR) and PHB synthase, which all together convert acetyl-CoA into PHB. (lu.se)
Inhibition2
- Differences in statin structure and binding characteristics may partially contribute to differences in potency of HMG-CoA reductase inhibition and other pharmacologic properties. (nih.gov)
- Inhibition of HMG-CoA reductase by statin in effect prevents the synthesis of mevalonic acid, a precursor of nonsteroidal isoprenoids, lipid attachment molecules for small G proteins, such as Ras, Rho, and Rac. (cdc.gov)
Reaction1
- Catalysis of the reaction: (S)-3-hydroxyacyl-CoA + NAD+ = 3-oxoacyl-CoA + NADH + H+. (yeastrc.org)
Metabolism1
- The formation of propionyl CoA in human metabolism is derived from many sources, chiefly catabolism of a number of essential amino acids (isoleucine, valine, threonine, methionine). (medscape.com)
Moiety1
- The arrangement between the flavin ring and deprotonated 3S-C8-CoA is consistent with a charge transfer interaction with the negatively charged acyl-chain of 3S-C8-CoA as an electron donor stacking on the pyrimidine moiety of the flavin ring as an electron acceptor. (rcsb.org)
Chairperson3
- COA Chairperson Gamaliel A. Cordoba, Commissioner Roland Café Pondoc and Assistant Commissioner Fortunata M. Rubico, Chair of the Technical Working Group (TWG) of the COA GFPS, led the COA contingent in accepting the awards. (businessmirror.com.ph)
- In fact, COA has issued Memorandum No. 2009-020 providing for Guidelines in the Conduct of the Audit of GAD Funds and Activities in Government Agencies three months before the Magna Carta of Women was signed into law on August 14, 2009," Chairperson Cordoba observed. (businessmirror.com.ph)
- 2 May 2011, submits the curriculum vitae of Ms Maria Gracia M. Pulido-Tan, who was appointed Chairperson of the Philippine Commission on Audit (COA) on 5 April 2011. (who.int)
Gene1
- Variants in the ethylmalonyl-CoA decarboxylase (ECHDC1) gene: a novel player in ethylmalonic aciduria? (nih.gov)
20201
- 59(3): 310-321, 2020 05 01. (bvsalud.org)
Pathway1
- Catalyzes a step in the 3-hydroxypropanoate/4-hydroxybutyrate cycle, an autotrophic CO2 fixation pathway found in some thermoacidophilic archaea. (expasy.org)
Defects1
- [ 1 , 2 ] Loss of mitochondria occurs in many of these maladies, but defects in the remaining mitochondria are emerging as key players in diabetes [ 3 ] and aging-related dysfunctions. (medscape.com)
Commission on Au1
- The Supreme Court Public Information Office shall upload a copy of G.R. No. 261207, Topbest Printing Corporation v. Commission on Audit , once it receives the same from the Office of the Clerk of Court En Banc. (gov.ph)
Enzymes3
- Arylamine N-acetyltransferases (NATs) are xenobiotic metabolizing enzymes that catalyze the acetyl-CoA-dependent acetylation of arylamines. (rcsb.org)
- Therefore, in contrast to previous data, this study shows that different orthologous NATs can bind their cofactors in a similar way, suggesting that the mode of binding CoA in this family of enzymes is less diverse than previously thought. (rcsb.org)
- Moreover, it supports the notion that the presence of the `mammalian/eukaryotic insertion loop' in certain NAT enzymes impacts the mode of binding CoA by imposing structural constraints. (rcsb.org)
Utilization1
- Productive utilization of CO 2 relies on a set of oxygen sensitive metalloenzymes exploiting the metal organic chemistry of nickel and cobalt to synthesize acetyl-CoA from activated one-carbon compounds. (degruyter.com)
Myopathy1
- HMG-CoA reductase inhibitor-induced myopathy in the rat: cyclosporine A interaction and mechanism studies. (aspetjournals.org)
Defect1
- In 1971, subsequent studies by Hsia et al of the original patient's sister demonstrated a specific defect in propionyl CoA carboxylase. (medscape.com)
Protein1
- Obviously, enhanced dietary protein intake has the same net effect by flooding the mitochondrion with propionyl CoA. (medscape.com)
Compounds2
- Without any functional 3-methylglutaconyl-CoA hydratase, leucine is not properly broken down, which leads to a buildup of related compounds, including multiple acids: 3-methylglutaconic acid, 3-methylglutaric acid, and 3-hydroxyisovaleric acid. (medlineplus.gov)
- 3-oxopalmitoyl-CoA, also known as 3-oxohexadecanoyl-CoA, belongs to the class of organic compounds known as long-chain 3-oxoacyl coas. (ymdb.ca)
Carbon1
- Accumulation of the 3-carbon fatty acyl-CoA within the mitochondrion leads to decreased free CoA for other reactions, which is alleviated by conversion of propionyl CoA to propionyl-carnitine. (medscape.com)
Clinical1
- The clinical diagnosis is confirmed health centres and 320 primary health was confirmed by chromosome analy- by chromosome analysis ( 3 ). (who.int)
20021
- 3(9): 1312-7, 2002 Sep. (bvsalud.org)
Homo1
- Importantly, it was found that the mode of binding of CoA is highly similar to that of M. marinum NAT1 but different from the modes reported for Bacillus anthracis NAT1 and Homo sapiens NAT2. (rcsb.org)
20011
- 2001;50(No. RR-3):[inclusive page numbers]. (cdc.gov)
Species1
- Background: Poly-3-d-hydroxybutyrate (PHB) that is a promising precursor for bioplastic with similar physical properties as polypropylene, is naturally produced by several bacterial species. (lu.se)
Acid3
- It derives from a benzoyl-CoA and a 3-hydroxybenzoic acid. (wikipedia.org)
- The structure of the model complex between lumiflavin and the deprotonated ethylthioester of 3-thiabutanoic acid was optimized by molecular orbital calculations. (rcsb.org)
- Other sources of propionyl CoA include odd chain-length fatty acids and the side chain of cholesterol, although these probably contribute very little in relation to the amino acid sources. (medscape.com)
Asymptomatic1
- He sat alone at 7.5 months, develop motor disease without overt crisis was crawling and pulling to stand at 8 and other biochemically affected individu- months and by 10 months he had 1 or 2 als remain asymptomatic [ 3-8 ]. (who.int)
View1
- view 3 months history. (flightaware.com)
Activity1
- Predicted to enable carboxy-lyase activity and enoyl-CoA hydratase activity. (nih.gov)