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AGXT2 | alanine--glyoxylate aminotransferase 2; 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 (514 aa) | |||
EHHADH | enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase (723 aa) | |||
MCEE | methylmalonyl CoA epimerase (176 aa) | |||
HIBADH | 3-hydroxyisobutyrate dehydrogenase (336 aa) | |||
MCCC1 | methylcrotonoyl-CoA carboxylase 1 (alpha) (725 aa) | |||
ABAT | 4-aminobutyrate aminotransferase; 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 (500 aa) | |||
MUT | methylmalonyl CoA mutase; Involved in the degradation of several amino acids, odd- chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species (750 aa) | |||
BCKDHB | branched chain keto acid dehydrogenase E1, beta polypeptide; 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) (392 aa) | |||
ACSF3 | acyl-CoA synthetase family member 3; Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates (576 aa) | |||
MDH2 | malate dehydrogenase 2, NAD (mitochondrial) (338 aa) | |||
HLCS | holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase); Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase (726 aa) | |||
MCCC2 | methylcrotonoyl-CoA carboxylase 2 (beta) (563 aa) | |||
ALDH3A2 | aldehyde dehydrogenase 3 family, member A2; Catalyzes the oxidation of long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1-phosphate (S1P) degradation product hexadecenal to hexadecenoic acid (508 aa) | |||
ALDH9A1 | aldehyde dehydrogenase 9 family, member A1; Converts gamma-trimethylaminobutyraldehyde into gamma- butyrobetaine. Catalyzes the irreversible oxidation of a broad range of aldehydes to the corresponding acids in an NAD-dependent reaction (518 aa) | |||
AOX1 | aldehyde oxidase 1 (1338 aa) | |||
PCCA | propionyl CoA carboxylase, alpha polypeptide (728 aa) | |||
ALDH1B1 | aldehyde dehydrogenase 1 family, member B1; ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation (517 aa) | |||
HADHA | hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; Bifunctional subunit (763 aa) | |||
PC | pyruvate carboxylase; Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate (1178 aa) | |||
HADH | hydroxyacyl-CoA dehydrogenase; Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA (331 aa) | |||
ALDH7A1 | aldehyde dehydrogenase 7 family, member A1; Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism (539 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) | |||
PCCB | propionyl CoA carboxylase, beta polypeptide (559 aa) | |||
ECHDC1 | enoyl CoA hydratase domain containing 1 (307 aa) | |||
ENSG00000257767 | Uncharacterized protein (245 aa) | |||
ALDH6A1 | aldehyde dehydrogenase 6 family, member A1; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA (535 aa) |