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| BDH1 | D-beta-hydroxybutyrate dehydrogenase, mitochondrial; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (344 aa) | ||||
| ACSM1 | Acyl-coenzyme A synthetase ACSM1, mitochondrial; Catalyzes the activation of fatty acids by CoA to produce an acyl-CoA, the first step in fatty acid metabolism. Capable of activating medium-chain fatty acids (e.g. butyric (C4) to decanoic (C10) acids), and certain carboxylate- containing xenobiotics, e.g. benzoate. Also catalyzes the activation of lipoate to lipoyl- nucleoside monophosphate. Activates lipoate with GTP at a 1000-fold higher rate than with ATP and activates both (R)- and (S)-lipoate to the respective lipoyl-GMP, with a preference for (R)- lipoate ; Belongs to the ATP-dep [...] (577 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) | ||||
| 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 (By similarity); Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (500 aa) | ||||
| AACS | Acetoacetyl-CoA synthetase. (672 aa) | ||||
| ACSM3 | Acyl-CoA synthetase medium chain family member 3. (581 aa) | ||||
| GAD1 | Glutamate decarboxylase 1; Catalyzes the production of GABA; Belongs to the group II decarboxylase family. (594 aa) | ||||
| GAD2 | Glutamate decarboxylase 2. (585 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) | ||||
| 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) | ||||
| HMGCLL1 | 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1. (340 aa) | ||||
| L2HGDH | L-2-hydroxyglutarate dehydrogenase, mitochondrial. (463 aa) | ||||
| ACSM2B | ACSM2A protein. (550 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) | ||||
| ALDH5A1 | Succinate-semialdehyde dehydrogenase; Belongs to the aldehyde dehydrogenase family. (526 aa) | ||||
| EHHADH | Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase. (723 aa) | ||||
| ACSM4 | Acyl-CoA synthetase medium chain family member 4. (580 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) | ||||
| LOC529488 | Uncharacterized protein. (628 aa) | ||||
| ACSM5 | Acyl-CoA synthetase medium chain family member 5. (590 aa) | ||||