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| ECI2 | Enoyl-CoA delta isomerase 2. (415 aa) | ||||
| ALDH6A1 | Methylmalonate-semialdehyde dehydrogenase acylating, mitochondrial isoform 1. (534 aa) | ||||
| ETFB | Electron transfer flavoprotein subunit beta; Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF- ubiquinone oxidoreductase. Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism. ETFB binds an AMP molecule that probably has a purely structural role. (255 aa) | ||||
| ECHDC1 | Ethylmalonyl-CoA decarboxylase 1; Belongs to the enoyl-CoA hydratase/isomerase family. (306 aa) | ||||
| ACAA2 | Uncharacterized protein; Belongs to the thiolase-like superfamily. Thiolase family. (398 aa) | ||||
| HADHB | Uncharacterized protein; Belongs to the thiolase-like superfamily. Thiolase family. (511 aa) | ||||
| HADHA | Trifunctional enzyme subunit alpha, mitochondrial; Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional en [...] (763 aa) | ||||
| ACAA1 | Acetyl-CoA acyltransferase 1; Belongs to the thiolase-like superfamily. Thiolase family. (432 aa) | ||||
| ACADL | Long-chain specific acyl-CoA dehydrogenase, mitochondrial; Long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA (By similarity). Among the different mitochondrial acyl-CoA dehydrogenas [...] (430 aa) | ||||
| HSD17B4 | Peroxisomal multifunctional enzyme type 2 isoform 2. (737 aa) | ||||
| EHHADH | Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase; Belongs to the enoyl-CoA hydratase/isomerase family. (731 aa) | ||||
| HSD17B10 | Hydroxysteroid 17-beta dehydrogenase 10; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (293 aa) | ||||
| ACSS3 | Acyl-CoA synthetase short-chain family member 3, mitochondrial isoform 1. (686 aa) | ||||
| ETFA | Electron transfer flavoprotein subunit alpha. (369 aa) | ||||
| ACOX1 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (660 aa) | ||||
| ACADS | Short-chain specific acyl-CoA dehydrogenase, mitochondrial; Short-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, short-chai [...] (612 aa) | ||||
| ECHS1 | Enoyl-CoA hydratase, mitochondrial; Belongs to the enoyl-CoA hydratase/isomerase family. (328 aa) | ||||
| ACOX2 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (681 aa) | ||||
| ACSL1 | Acyl-CoA synthetase long chain family member 1. (771 aa) | ||||
| ACADSB | Acyl-CoA dehydrogenase short/branched chain. (432 aa) | ||||
| PCCA | Propionyl-CoA carboxylase alpha chain, mitochondrial; This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domai [...] (730 aa) | ||||
| HADH | Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3- hydroxybutyryl-CoA; Belongs to the 3-hydroxyacyl-CoA dehydrogenase family. (314 aa) | ||||
| GCDH | Glutaryl-CoA dehydrogenase, mitochondrial; Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L- hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. (438 aa) | ||||
| ETFDH | Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; Accepts electrons from ETF and reduces ubiquinone. (615 aa) | ||||
| ACOX3 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (720 aa) | ||||
| PCCB | Propionyl-CoA carboxylase beta chain, mitochondrial; This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain [...] (558 aa) | ||||
| ACADM | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; Acyl-CoA dehydrogenase specific for acyl chain lengths of 4 to 16 that catalyzes the initial step of fatty acid beta-oxidation. Utilizes the electron transfer flavoprotein (ETF) as an electron acceptor to transfer electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). (454 aa) | ||||
| ACADVL | Acyl-CoA dehydrogenase very long chain. (681 aa) | ||||
| CPT2 | Carn_acyltransf domain-containing protein; Belongs to the carnitine/choline acetyltransferase family. (658 aa) | ||||
| HSDL2 | Hydroxysteroid dehydrogenase like 2. (418 aa) | ||||