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ALDH5A1 | Succinate-semialdehyde dehydrogenase; Belongs to the aldehyde dehydrogenase family. (535 aa) | ||||
HMGCLL1 | 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1. (425 aa) | ||||
ACSM3 | Acyl-CoA synthetase medium chain family member 3. (581 aa) | ||||
ACSM2B | Uncharacterized protein. (578 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) | ||||
AACS | Acetoacetyl-CoA synthetase. (795 aa) | ||||
GAD2 | Glutamate decarboxylase 2; Catalyzes the production of GABA. (584 aa) | ||||
BDH1 | D-beta-hydroxybutyrate dehydrogenase, mitochondrial; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (346 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. (576 aa) | ||||
ACSM5 | Acyl-coenzyme A synthetase ACSM5, mitochondrial isoform 1. (579 aa) | ||||
BDH2 | 3-hydroxybutyrate dehydrogenase type 2 isoform X2. (245 aa) | ||||
EHHADH | Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase; Belongs to the enoyl-CoA hydratase/isomerase family. (731 aa) | ||||
ACAT1 | Acetyl-CoA acetyltransferase 1; Belongs to the thiolase-like superfamily. Thiolase family. (426 aa) | ||||
ACSM4 | Acyl-CoA synthetase medium chain family member 4. (580 aa) | ||||
L2HGDH | L-2-hydroxyglutarate dehydrogenase. (463 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) | ||||
ABAT | 4-aminobutyrate aminotransferase, mitochondrial; Catalyzes the conversion of gamma-aminobutyrate and L-beta- aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (520 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) | ||||
GAD1 | Glutamate decarboxylase 1; Catalyzes the production of GABA; Belongs to the group II decarboxylase family. (728 aa) | ||||
ACAT2 | Acetyl-CoA acetyltransferase 2; Belongs to the thiolase-like superfamily. Thiolase family. (447 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) | ||||
OXCT1 | Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; 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. (562 aa) |