The conversion of malonic acid to malonyl-CoA by acyl-CoA synthetase represents the first step in the mitochondrial fatty acid ... such as pyruvate dehydrogenase complex (PDHC) and α-ketoglutarate dehydrogenase complex (α-KGDHC), among others. This ... At this point, the acyl-CoA synthease encoded by ACSF3 could convert the methylmalonic acid back to methylmalonyl-CoA and then ... Malonyl-CoA decarboxylase acts as a catalyst in the conversion of malonyl-CoA to acetyl-CoA and CO2. It is speculated that in ...

Glutamate-5-semialdehyde is first formed by glutamate 5-kinase (ATP-dependent) and glutamate-5-semialdehyde dehydrogenase ( ... to the Acyl Carrier Protein (ACP) by a KS domain, followed by transfer to malonyl-ACP via decarboxylative Claisen condensation ... which is synthesized from glycine and succinyl-CoA. ALA dehydratase catalyzes the condensation of two ALA molecules via a Knorr ...

... and the acyl carbon (X). This class of hydrogenases thus provides examples of naturally occurring iron acyl complexes. ... Carbon monoxide dehydrogenase (CODH) catalyzes the water-gas shift reaction, which provides CO (through a nickelacarboxylate ... The latter step is effected by the Ni-Fe enzyme CO-methylating acetyl-CoA synthase (ACS). CODH and ACS often occur together in ... February 2009). "The crystal structure of C176A mutated [Fe]-hydrogenase suggests an acyl-iron ligation in the active site iron ...

2-hydroxyisobutanoyl-CoA mutase EC 5.3.3.21: Δ3,5-Δ2,4-dienoyl-CoA isomerase * EC 5.3.3.22: lutein isomerase * EC 5.3.3.23: S- ... acyl-carrier protein] isomerase EC 5.3.3.15: Now EC 5.3.2.7, ascopyrone tautomerase EC 5.3.3.16: Now EC 5.3.2.8, 4- ... 11-cis-retinol dehydrogenase. EC 5.2.1.4: maleylpyruvate isomerase EC 5.2.1.5: linoleate isomerase EC 5.2.1.6: furylfuramide ... ethylmalonyl-CoA mutase * EC 5.4.99.64: 2-hydroxyisobutanoyl-CoA mutase * EC 5.4.99.65: pre-α-onocerin synthase * EC 5.4.99.66 ...

... oleoyl-CoA + 2 ferricytochrome b5 + H2O EC 1.14.19.3 linoleoyl-CoA 9-desaturase linoleoyl-CoA + 2 ferrocytochrome b5 + O2 + 2 ... L-lactate dehydrogenase; EC 1.1.2.3), sulfite oxidase (EC 1.8.3.1), plant and fungal nitrate reductases (EC 1.7.1.1, EC 1.7.1.2 ... EC 1.7.1.3), and plant and fungal cytochrome b5/acyl lipid desaturase fusion proteins. 3-D structures of a number of cytochrome ... H2O EC 1.14.19.1 stearoyl-CoA 9-desaturase stearoyl-CoA + 2 ferrocytochrome b5 + O2 + 2 H+ → ...

3-Hydroxyisovaleryl CoA accumulation can inhibit cellular respiration either directly or via effects on the ratios of acyl CoA: ... whereas the dehydrogenase enzyme is found exclusively in the mitochondrion (Sabourin and Bieber 1981, 1983). Importantly, this ... Metabolic impairment diverts methylcrotonyl CoA to 3-hydroxyisovaleryl CoA in a reaction catalyzed by enoyl-CoA hydratase (22, ... MC-CoA). It can be metabolized into HMB, MC-CoA, and HMG-CoA in humans. This reaction is catalyzed by an unknown thioesterase ...

PCC not only catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA, but also acts on several different acyl-CoAs. ... For example, inhibition of pyruvate dehydrogenase by an accumulation of propionyl-CoA in Rhodobacter sphaeroides can prove ... methylmalonyl-CoA by methylmalonyl-CoA racemase. (R)-Methylmalonyl-CoA is converted to succinyl-CoA, an intermediate in the ... Because of the structure of propionyl-CoA, Gen5 distinguishes between different acyl-CoA molecules. In fact, it was found that ...

Cardiac ischemia, as it slows the oxidation of fatty acids, causes an accumulation of acyl-CoA and induces KATP channel opening ... Ardehali H, Chen Z, Ko Y, Mejía-Alvarez R, Marbán E (August 2004). "Multiprotein complex containing succinate dehydrogenase ... Cardiomyocytes, on the other hand, derive the majority of their energy from long-chain fatty acids and their acyl-CoA ... More recently, it was discovered that certain multiprotein complexes containing succinate dehydrogenase can provide activity ...

... through substrate-level phosphorylation catalyzed by succinyl-CoA synthetase, as succinyl-CoA is converted to succinate, three ... Another malate dehydrogenase-catalyzed reaction occurs in the opposite direction, producing oxaloacetate and NADH from the ... Dozens of ATP equivalents are generated by the beta-oxidation of a single long acyl chain. In oxidative phosphorylation, the ... The acetyl-CoA is metabolized by the citric acid cycle to generate ATP, while the NADH and FADH2 are used by oxidative ...

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 ...

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 ...

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 ...

... aldehyde dehydrogenase, alcohol dehydrogenase, and phosphotransacylase. Because aldehydes can be toxic to cells and/or volatile ... Ethanol is likely a lost carbon source, but acetyl-phosphate can either generate ATP or be recycled to acetyl-CoA and enter the ... resulting in a phosphorylated acyl compound that can readily be a source of substrate-level phosphorylation or enter central ... The aldehyde is initially fixed to coenzyme A by a NAD+-dependent aldehyde dehydrogenase, but these two cofactors must be ...

Malonyl CoA is formed there from acetyl CoA by the action of acetyl-CoA carboxylase, and the malonate is transferred to an acyl ... Malonic acid is the classic example of a competitive inhibitor of the enzyme succinate dehydrogenase (complex II), in the ... malonyl-CoA, is an important precursor in cytosolic fatty acid biosynthesis along with acetyl CoA. ... in which it is converted to malonyl-CoA by malonyl-CoA synthetase (ACSF3). Additionally, the coenzyme A derivative of malonate ...

ADP-dependent short-chain-acyl-CoA hydrolase EC 3.1.2.19: ADP-dependent medium-chain-acyl-CoA hydrolase EC 3.1.2.20: acyl-CoA ... pyruvate dehydrogenase (acetyl-transferring)]-phosphatase EC 3.1.3.44: [acetyl-CoA carboxylase]-phosphatase EC 3.1.3.45: 3- ... acetyl-CoA hydrolase EC 3.1.2.2: palmitoyl-CoA hydrolase EC 3.1.2.3: succinyl-CoA hydrolase EC 3.1.2.4: 3-hydroxyisobutyryl-CoA ... EC 3.1.2.25: phenylacetyl-CoA hydrolase EC 3.1.2.26: Now EC 2.8.3.25, bile acid CoA transferase EC 3.1.2.27: choloyl-CoA ...

An example of this are the dehydrogenases that use nicotinamide adenine dinucleotide (NAD+) as a cofactor. Here, hundreds of ... Denessiouk KA, Rantanen VV, Johnson MS (August 2001). "Adenine recognition: a motif present in ATP-, CoA-, NAD-, NADP-, and FAD ... The nucleotide adenosine is present in cofactors that catalyse many basic metabolic reactions such as methyl, acyl, and ... For example, the multienzyme complex pyruvate dehydrogenase at the junction of glycolysis and the citric acid cycle requires ...

In the hepatic PEMT route, 3-phosphoglycerate (3PG) receives 2 acyl groups from acyl-CoA forming a phosphatidic acid. It reacts ... This is oxidized by mitochondrial or cytosolic betaine-aldehyde dehydrogenases to trimethylglycine. Trimethylglycine is a ...

ECH is used in β-oxidation to add a hydroxyl group and a proton to the unsaturated β-carbon on a fatty-acyl CoA. ECH functions ... whereas the dehydrogenase enzyme is found exclusively in the mitochondrion (Sabourin and Bieber 1981, 1983). Importantly, this ... The two amino acids hold a water molecule in place, allowing it to attack in a syn addition to an α-β unsaturated acyl-CoA at ... The α-carbon then grabs another proton, which completes the formation of the beta-hydroxy acyl-CoA. It is also known from ...

Acetyl-coA inhibits pyruvate dehydrogenase, while succinyl-CoA inhibits alpha-ketoglutarate dehydrogenase and citrate synthase ... Acyl-CoA is oxidized to trans-Enoyl-CoA while FAD is reduced to FADH2, which is similar to the oxidation of succinate to ... inhibits pyruvate dehydrogenase, isocitrate dehydrogenase, α-ketoglutarate dehydrogenase, and also citrate synthase. ... Lastly, beta-hydroxyacyl-CoA is oxidized to beta-ketoacyl-CoA while NAD+ is reduced to NADH, which follows the same process as ...

3-methylglutaryl-CoA, and 3-hydroxy-3-methylglutaryl-CoA that act as reactive acyl species on MCC in a negative feedback loop. ... whereas the dehydrogenase enzyme is found exclusively in the mitochondrion (Sabourin and Bieber 1981, 1983). Importantly, this ... 3-Methylglutaconyl CoA is then hydrated to produce 3-hydroxy-3-methylglutaryl CoA. 3-Hydroxy-3-methylglutaryl CoA is cleaved ... Methylcrotonyl CoA carboxylase (EC 6.4.1.4, MCC) (3-methylcrotonyl CoA carboxylase, methylcrotonoyl-CoA carboxylase) is a ...

This system is distinct from the branched-chain fatty acid synthetase that utilizes short-chain acyl-CoA esters as primers. α- ... the cycle as CO2 in the decarboxylation reactions catalyzed by isocitrate dehydrogenase and alpha-ketoglutarate dehydrogenase. ... Regulation Acetyl-CoA is formed into malonyl-CoA by acetyl-CoA carboxylase, at which point malonyl-CoA is destined to feed into ... and isoleucine to form 2-methylpropanyl-CoA, 3-methylbutyryl-CoA, and 2-methylbutyryl-CoA, respectively. 2-Methylpropanyl-CoA ...

... on NADPH dehydrogenase ("old yellow enzyme"), David P. Ballou, and Paul Engel on butyryl-CoA dehydrogenase, among others. I.C.I ... Engel, Paul C.; Massey, V. (1971). "Green butyryl-coenzyme a dehydrogenase. An enzyme-acyl-coenzyme a complex". Biochemical ... Moore, E G; Cardemil, E; Massey, V (1978). "Production of a covalent flavin linkage in lipoamide dehydrogenase - reaction with ... Singer, Thomas P.; Kearney, Edna B.; Massey, Vincent (1956). "Observations on the flavin moiety of succinic dehydrogenase". ...

This malyl-CoA intermediate then undergoes hydrolysis at the acyl-CoA portion, generating a carboxylate anion. The enzyme ... This operon contains genes coding for malate synthase (aceB), isocitrate lyase (aceA), and isocitrate dehydrogenase kinase/ ... CoA The 3 substrates of this enzyme are acetyl-CoA, H2O, and glyoxylate, whereas its two products are (S)-malate and CoA. This ... it is thought to convert citramalyl-CoA into pyruvate and acetyl-CoA. Without this conversion, itaconyl-CoA, a precursor to ...

The fatty acids are synthesized by a series of decarboxylative Claisen condensation reactions from acetyl-CoA and malonyl-CoA. ... Anselmi C, Grininger M, Gipson P, Faraldo-Gómez JD (September 2010). "Mechanism of substrate shuttling by the acyl-carrier ... short-chain dehydrogenase/reductase and related enzymes) nomenclature initiative". Chemico-Biological Interactions. 178 (1-3): ... from acetyl-CoA and malonyl-CoA, in the presence of NADPH. ... acyl carrier protein (ACP) and thioesterase (TE)). The ...

... and the regulation of pyruvate dehydrogenase (PDH), which converts pyruvate to acetyl CoA. Transferases are also utilized ... Transfer of acyl groups or acyl groups that become alkyl groups during the process of being transferred are key aspects of EC ... Succinyl-CoA:3-ketoacid CoA transferase deficiency (or SCOT deficiency) leads to a buildup of ketones.Ketones are created upon ... "Succinyl-CoA:3-ketoacid CoA transferase deficiency". Genetics Home Reference. National Institute of Health. Retrieved 4 ...

... fatty acyl CoA acyltransferase and sulfoquinovosyl glyceride: fatty acyl ester hydrolase pathways". Journal of Lipid Research. ... catalyzed by sulfolactaldehyde dehydrogenase using NAD+ as a co-factor). The transaldolase can also catalyze transfer of the C3 ... catalyzed by sulfolactaldehyde dehydrogenase with NAD+ co-factor). The major steps in the SFT pathway are: isomerization of ... catalyzed by sulfoquinovose dehydrogenase with NAD+ co-factor); hydrolysis of sulfogluconolactone to sulfogluconate acid ( ...

Acetate-CoA ligase EC 6.2.1.2: Medium-chain acyl-CoA ligase EC 6.2.1.3: Long-chain-fatty-acid-CoA ligase EC 6.2.1.4: Succinate- ... EC 1.1.1.10 Lactate dehydrogenase EC 1.1.1.27 Malate dehydrogenase EC 1.1.1.37 Isocitrate dehydrogenase EC 1.1.1.42 HMG-CoA ... Glutarate-CoA ligase EC 6.2.1.7: Cholate-CoA ligase EC 6.2.1.8: Oxalate-CoA ligase EC 6.2.1.9: Malate-CoA ligase EC 6.2.1.10: ... Acid-CoA ligase (GDP-forming) EC 6.2.1.11: Biotin-CoA ligase EC 6.2.1.12: 4-coumarate-CoA ligase EC 6.2.1.13: Acetate-CoA ...

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 ...

PRKAG1 Senses energy level and inactivates HMGCoA reductase and Acetyl CoA Carboxylase PRKAA1 NM_006251 Catalytic subunit of ... macrophage migration inhibitory factor TAPBP ADRBK1 can downregulate response to epinephrine AGPAT1 acyl 3 phosphoglycerol acyl ... subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is ... 002494Homo sapiens NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa NDUFC2 NM_004549 NDUFC2-KCTD14 NM_001203260 ...

Isovaleryl-CoA is subsequently metabolized by isovaleryl-CoA dehydrogenase and converted to MC-CoA, which is used in the ... 3-Hydroxyisovaleryl CoA accumulation can inhibit cellular respiration either directly or via effects on the ratios of acyl CoA: ... MG-CoA), which is subsequently converted to HMG-CoA by methylglutaconyl-CoA hydratase. HMG-CoA is then cleaved into acetyl-CoA ... producing β-methylcrotonyl-CoA (MC-CoA) or hydroxymethylglutaryl-CoA (HMG-CoA) respectively. MC-CoA is then converted by the ...