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| BDH1 | D-beta-hydroxybutyrate dehydrogenase, mitochondrial; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (344 aa) | ||||
| HADH | Hydroxyacyl-CoA dehydrogenase. (314 aa) | ||||
| BDH2 | 3-hydroxybutyrate dehydrogenase type 2; Dehydrogenase that mediates the formation of 2,5- dihydroxybenzoic acid (2,5-DHBA), a siderophore that shares structural similarities with bacterial enterobactin and associates with LCN2, thereby playing a key role in iron assimilation and homeostasis. Plays a role in susceptibility to bacterial infection by providing an assimilable source of iron that is exploited by pathogenic bacteria (By similarity). Also acts as a 3-hydroxybutyrate dehydrogenase (By similarity). (245 aa) | ||||
| ACAT2 | Acetyl-Coenzyme A acetyltransferase 2; Belongs to the thiolase-like superfamily. Thiolase family. (397 aa) | ||||
| ECHDC2 | Enoyl-CoA hydratase domain-containing protein 2, mitochondrial. (346 aa) | ||||
| ACADVL | Very long-chain specific acyl-CoA dehydrogenase, mitochondrial; Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, myristoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons; Belongs to the acyl-CoA dehydrogenase family. (655 aa) | ||||
| HMGCS2 | Hydroxymethylglutaryl-CoA synthase, mitochondrial; This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase; Belongs to the thiolase-like superfamily. HMG-CoA synthase family. (508 aa) | ||||
| ACOX2 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (682 aa) | ||||
| AACS | Acetoacetyl-CoA synthetase. (672 aa) | ||||
| HSD17B4 | Hydroxysteroid (17-beta) dehydrogenase 4. (736 aa) | ||||
| HMGCR | 3-hydroxy-3-methylglutaryl-coenzyme A reductase; Transmembrane glycoprotein that is the rate-limiting enzyme in cholesterol biosynthesis as well as in the biosynthesis of nonsterol isoprenoids that are essential for normal cell function including ubiquinone and geranylgeranyl proteins; Belongs to the HMG-CoA reductase family. (888 aa) | ||||
| ECI1 | Enoyl-CoA delta isomerase 1. (303 aa) | ||||
| ACADL | Acyl-Coenzyme A dehydrogenase, long chain. (430 aa) | ||||
| HMGCS1 | 3-hydroxy-3-methylglutaryl coenzyme A synthase; This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. (571 aa) | ||||
| ETFA | Electron transfer flavoprotein subunit alpha, mitochondrial; 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 (ETF dehydrogenase). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism. (333 aa) | ||||
| ACAT1 | Acetyl-CoA acetyltransferase, mitochondrial; This is one of the enzymes that catalyzes the last step of the mitochondrial beta-oxidation pathway, an aerobic process breaking down fatty acids into acetyl-CoA. Using free coenzyme A/CoA, catalyzes the thiolytic cleavage of medium- to long-chain 3-oxoacyl-CoAs into acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms. The activity of the enzyme is reversible and it can also catalyze the condensation of two acetyl-CoA molecules into acetoacetyl-CoA. Thereby, it plays a major role in ketone body metabolism. Belongs to the thiolase-l [...] (422 aa) | ||||
| ACAD8 | Isobutyryl-CoA dehydrogenase, mitochondrial; Isobutyryl-CoA dehydrogenase which catalyzes one of the steps of the valine catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA. (416 aa) | ||||
| CPT2 | Carnitine O-palmitoyltransferase 2, mitochondrial; Belongs to the carnitine/choline acetyltransferase family. (658 aa) | ||||
| HMGCLL1 | 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1. (340 aa) | ||||
| ACADSB | Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2- methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent (By similarity). (432 aa) | ||||
| ACAA1 | Mitochondrial acetyl-Coenzyme A acyltransferase 1; Belongs to the thiolase-like superfamily. Thiolase family. (424 aa) | ||||
| DECR1 | 2,4-dienoyl-CoA reductase 1. (329 aa) | ||||
| AMACR | Alpha-methylacyl-CoA racemase. (382 aa) | ||||
| HMGCL | Hydroxymethylglutaryl-CoA lyase, mitochondrial; Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3- methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta- hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism. (325 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). (421 aa) | ||||
| ACOX3 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (700 aa) | ||||
| HSDL2 | Hydroxysteroid dehydrogenase-like protein 2; Has apparently no steroid dehydrogenase activity. Belongs to the short-chain dehydrogenases/reductases (SDR) family. (418 aa) | ||||
| EHHADH | Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase. (723 aa) | ||||
| CARTPT | Cocaine- and amphetamine-regulated transcript protein; Satiety factor closely associated with the actions of leptin and neuropeptide y; this anorectic peptide inhibits both normal and starvation-induced feeding and completely blocks the feeding response induced by neuropeptide Y and regulated by leptin in the hypothalamus. Belongs to the CART family. (116 aa) | ||||
| ETFDH | Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; Accepts electrons from ETF and reduces ubiquinone. Belongs to the ETF-QO/FixC family. (873 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 (By similarity). Among the different mitochondrial acyl-CoA dehydrogen [...] (438 aa) | ||||
| OXCT1 | Succinyl-CoA:3-ketoacid-coenzyme A transferase; Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. (523 aa) | ||||
| ECH1 | Enoyl-CoA hydratase 1; Belongs to the enoyl-CoA hydratase/isomerase family. (326 aa) | ||||
| ECI2 | Enoyl-CoA delta isomerase 2. (454 aa) | ||||
| ENSBTAP00000063081 | Uncharacterized protein. (313 aa) | ||||
| CRYL1 | Lambda-crystallin homolog. (322 aa) | ||||
| AUH | Uncharacterized protein. (275 aa) | ||||
| LOC614914 | 4HBT domain-containing protein. (200 aa) | ||||
| HADHA | Hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha; Belongs to the enoyl-CoA hydratase/isomerase family. (771 aa) | ||||
| ACAA2 | 3-ketoacyl-CoA thiolase, mitochondrial; Belongs to the thiolase-like superfamily. Thiolase family. (455 aa) | ||||
| ENSBTAP00000070244 | Uncharacterized protein; Belongs to the thiolase-like superfamily. Thiolase family. (372 aa) | ||||
| ACOX1 | Peroxisomal acyl-CoA oxidase 1, A chain; Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl- CoAs. (660 aa) | ||||
| HADHB | Trifunctional enzyme subunit beta, 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 enz [...] (486 aa) | ||||
| ENSBTAP00000071610 | Acyl-CoA_dh_1 domain-containing protein. (216 aa) | ||||
| ECHS1 | Enoyl-CoA hydratase, mitochondrial; Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate (By similarity). Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate (By similarity); Belongs to the enoyl-CoA hydratase/isomerase family. (341 aa) | ||||