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| PCK2 | Phosphoenolpyruvate carboxykinase [GTP], mitochondrial; Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle; Belongs to the phosphoenolpyruvate carboxykinase [GTP] family (640 aa) | |||
| AGXT2 | Alanine--glyoxylate aminotransferase 2, mitochondrial; Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family (514 aa) | |||
| EHHADH | Peroxisomal bifunctional enzyme; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase; In the C-terminal section; belongs to the 3- hydroxyacyl-CoA dehydrogenase family (723 aa) | |||
| ACYP1 | Acylphosphatase-1; Its physiological role is not yet clear; Belongs to the acylphosphatase family (99 aa) | |||
| ACSS2 | Acetyl-coenzyme A synthetase, cytoplasmic; Activates acetate so that it can be used for lipid synthesis or for energy generation; Acyl-CoA synthetase family (714 aa) | |||
| MLYCD | Malonyl-CoA decarboxylase, mitochondrial; 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. Plays a role in the metabolic balance between glucose and lipid oxidation in mus [...] (493 aa) | |||
| HIBADH | 3-hydroxyisobutyrate dehydrogenase, mitochondrial; 3-hydroxyisobutyrate dehydrogenase (336 aa) | |||
| ACAT1 | Acetyl-CoA acetyltransferase, mitochondrial; Plays a major role in ketone body metabolism (427 aa) | |||
| DLAT | Dihydrolipoamide S-acetyltransferase; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle (647 aa) | |||
| ACAA2 | 3-ketoacyl-CoA thiolase, mitochondrial; Abolishes BNIP3-mediated apoptosis and mitochondrial damage (397 aa) | |||
| PDHA2 | Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle (388 aa) | |||
| ACOT12 | Acyl-coenzyme A thioesterase 12; Hydrolyzes acetyl-CoA to acetate and CoA; Acyl-CoA thioesterases (555 aa) | |||
| PDHB | Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle (359 aa) | |||
| ACSS1 | Acetyl-coenzyme A synthetase 2-like, mitochondrial; Important for maintaining normal body temperature during fasting and for energy homeostasis. Essential for energy expenditure under ketogenic conditions (By similarity). Converts acetate to acetyl-CoA so that it can be used for oxidation through the tricarboxylic cycle to produce ATP and CO(2); Belongs to the ATP-dependent AMP-binding enzyme family (689 aa) | |||
| PCK1 | Phosphoenolpyruvate carboxykinase, cytosolic [GTP]; Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle; Belongs to the phosphoenolpyruvate carboxykinase [GTP] family (622 aa) | |||
| HADHB | hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta (474 aa) | |||
| ACAA1 | 3-ketoacyl-CoA thiolase, peroxisomal; acetyl-CoA acyltransferase 1 (424 aa) | |||
| ACAT2 | Acetyl-CoA acetyltransferase, cytosolic; acetyl-CoA acetyltransferase 2 (397 aa) | |||
| DBT | Dihydrolipoamide branched chain transacylase E2; The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components- branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component (482 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) (454 aa) | |||
| PCCA | propionyl-CoA carboxylase alpha subunit (728 aa) | |||
| ACYP2 | Acylphosphatase-2; Its physiological role is not yet clear (99 aa) | |||
| PCCB | propionyl-CoA carboxylase beta subunit (559 aa) | |||
| ECHDC1 | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, a potentially toxic metabolite, to form butyryl-CoA, suggesting it might be involved in metabolite proofreading. Also has methylmalonyl-CoA decarboxylase activity at lower level (307 aa) | |||
| ACSS3 | Acyl-CoA synthetase short-chain family member 3, mitochondrial; Activates acetate so that it can be used for lipid synthesis or for energy generation; Belongs to the ATP-dependent AMP-binding enzyme family (686 aa) | |||
| ALDH6A1 | Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA; Aldehyde dehydrogenases (535 aa) | |||
