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| DAO | D-amino-acid oxidase; Regulates the level of the neuromodulator D-serine in the brain. Has high activity towards D-DOPA and contributes to dopamine synthesis. Could act as a detoxifying agent which removes D-amino acids accumulated during aging. Acts on a variety of D- amino acids with a preference for those having small hydrophobic side chains followed by those bearing polar, aromatic, and basic groups. Does not act on acidic amino acids; Belongs to the DAMOX/DASOX family (347 aa) | |||
| GCLC | Glutamate-cysteine ligase catalytic subunit (637 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) | |||
| 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) | |||
| SDS | L-serine dehydratase/L-threonine deaminase; Serine dehydratase (328 aa) | |||
| HIBADH | 3-hydroxyisobutyrate dehydrogenase, mitochondrial; 3-hydroxyisobutyrate dehydrogenase (336 aa) | |||
| BCKDHA | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 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) (445 aa) | |||
| LDHAL6A | L-lactate dehydrogenase A-like 6A; Displays an lactate dehydrogenase activity. Significantly increases the transcriptional activity of JUN, when overexpressed (332 aa) | |||
| AGXT | Alanine-glyoxylate aminotransferase (392 aa) | |||
| PIPOX | Peroxisomal sarcosine oxidase; Metabolizes sarcosine, L-pipecolic acid and L-proline (390 aa) | |||
| SHMT1 | Serine hydroxymethyltransferase, cytosolic; Interconversion of serine and glycine (483 aa) | |||
| ALAS2 | 5-aminolevulinate synthase, erythroid-specific, mitochondrial; 5’-aminolevulinate synthase 2 (587 aa) | |||
| SHMT2 | Serine hydroxymethyltransferase, mitochondrial; Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism. Thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA. Interconversion of serine and glycine. Associates with mitochondrial DNA. Plays a role in the deubiquitination of target proteins as component of the BRISC complex. Required for IFNAR1 deubiquitination by the BRISC complex; Belongs to the SHMT family (504 aa) | |||
| GCLM | Glutamate-cysteine ligase modifier subunit; Belongs to the aldo/keto reductase family. Glutamate-- cysteine ligase light chain subfamily (274 aa) | |||
| CTH | Cystathionine gamma-lyase; Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target protei [...] (405 aa) | |||
| SARDH | Sarcosine dehydrogenase, mitochondrial; Sarcosine dehydrogenase; Belongs to the GcvT family (918 aa) | |||
| GNMT | Glycine N-methyltransferase; Catalyzes the methylation of glycine by using S- adenosylmethionine (AdoMet) to form N-methylglycine (sarcosine) with the concomitant production of S-adenosylhomocysteine (AdoHcy). Possible crucial role in the regulation of tissue concentration of AdoMet and of metabolism of methionine; Seven-beta-strand methyltransferase motif containing (295 aa) | |||
| AOX1 | Aldehyde oxidase; Oxidase with broad substrate specificity, oxidizing aromatic azaheterocycles, such as N1-methylnicotinamide, N- methylphthalazinium and phthalazine, as well as aldehydes, such as benzaldehyde, retinal, pyridoxal, and vanillin. Plays a key role in the metabolism of xenobiotics and drugs containing aromatic azaheterocyclic substituents. Participates in the bioactivation of prodrugs such as famciclovir, catalyzing the oxidation step from 6-deoxypenciclovir to penciclovir, which is a potent antiviral agent. Is probably involved in the regulation of reactive oxygen species [...] (1338 aa) | |||
| GLDC | Glycine dehydrogenase (decarboxylating), mitochondrial; The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH) (1020 aa) | |||
| GCAT | 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial; Glycine C-acetyltransferase; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family (445 aa) | |||
| ALAS1 | 5-aminolevulinate synthase, nonspecific, mitochondrial; 5’-aminolevulinate synthase 1; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family (640 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; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family (500 aa) | |||
| GATM | Glycine amidinotransferase, mitochondrial; Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis; Belongs to the amidinotransferase family (423 aa) | |||
| ALDH6A1 | Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA; Aldehyde dehydrogenases (535 aa) | |||
| HADH | Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA; Belongs to the 3-hydroxyacyl-CoA dehydrogenase family (331 aa) | |||
