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OXCT1 | 3-oxoacid CoA transferase 1; 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 (By similarity) (520 aa) | |||
EHHADH | enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase (723 aa) | |||
ACADS | acyl-CoA dehydrogenase, C-2 to C-3 short chain (412 aa) | |||
ACSS2 | acyl-CoA synthetase short-chain family member 2; Activates acetate so that it can be used for lipid synthesis or for energy generation (714 aa) | |||
MLYCD | malonyl-CoA decarboxylase; Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids (493 aa) | |||
ACAT1 | acetyl-CoA acetyltransferase 1; Plays a major role in ketone body metabolism (427 aa) | |||
ACAA2 | acetyl-CoA acyltransferase 2; Abolishes BNIP3-mediated apoptosis and mitochondrial damage (397 aa) | |||
ACOX1 | acyl-CoA oxidase 1, palmitoyl (660 aa) | |||
ACOT12 | acyl-CoA thioesterase 12; Hydrolyzes acetyl-CoA to acetate and CoA (555 aa) | |||
ACSS1 | acyl-CoA synthetase short-chain family member 1 (689 aa) | |||
AACS | acetoacetyl-CoA synthetase (672 aa) | |||
HADHB | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit (474 aa) | |||
ACAA1 | acetyl-CoA acyltransferase 1 (424 aa) | |||
DLST | dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex); The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of 3 enzymatic components- 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (453 aa) | |||
CS | citrate synthase (466 aa) | |||
UBC | ubiquitin C (685 aa) | |||
ACOX3 | acyl-CoA oxidase 3, pristanoyl; Oxidizes the CoA-esters of 2-methyl-branched fatty acids (By similarity) (700 aa) | |||
ACAT2 | acetyl-CoA acetyltransferase 2 (397 aa) | |||
ACADSB | acyl-CoA dehydrogenase, short/branched chain; 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 (432 aa) | |||
OXCT2 | 3-oxoacid CoA transferase 2; 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 (By similarity) (517 aa) | |||
HMGCL | 3-hydroxymethyl-3-methylglutaryl-CoA lyase; Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism (325 aa) | |||
SUCLA2 | succinate-CoA ligase, ADP-forming, beta subunit; Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA (By similarity) (463 aa) | |||
HADHA | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; Bifunctional subunit (763 aa) | |||
ACADM | acyl-CoA dehydrogenase, C-4 to C-12 straight chain; This enzyme is specific for acyl chain lengths of 4 to 16 (425 aa) | |||
SUCLG2 | succinate-CoA ligase, GDP-forming, beta subunit; Catalyzes the GTP-dependent ligation of succinate and CoA to form succinyl-CoA (By similarity) (440 aa) | |||
ALDH6A1 | aldehyde dehydrogenase 6 family, member A1; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA (535 aa) |