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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) | ||||
ECHS1 | Enoyl-CoA hydratase, mitochondrial; Belongs to the enoyl-CoA hydratase/isomerase family. (328 aa) | ||||
ECH1 | Enoyl-CoA hydratase 1; Belongs to the enoyl-CoA hydratase/isomerase family. (368 aa) | ||||
CDYL2 | Chromodomain Y like 2. (539 aa) | ||||
HIBCH | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite; Belongs to the enoyl-CoA hydratase/isomerase family. (386 aa) | ||||
ECHDC2 | Enoyl-CoA hydratase domain containing 2; Belongs to the enoyl-CoA hydratase/isomerase family. (335 aa) | ||||
CDYL | Chromodomain Y like. (568 aa) | ||||
ECI2 | Enoyl-CoA delta isomerase 2. (415 aa) | ||||
ECHDC1 | Ethylmalonyl-CoA decarboxylase 1; Belongs to the enoyl-CoA hydratase/isomerase family. (306 aa) | ||||
AUH | Methylglutaconyl-CoA hydratase, mitochondrial isoform X1; Belongs to the enoyl-CoA hydratase/isomerase family. (338 aa) |