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| ASNS | Asparagine synthetase (561 aa) | |||
| MTHFD1 | C-1-tetrahydrofolate synthase, cytoplasmic; Methylenetetrahydrofolate dehydrogenase, cyclohydrolase and formyltetrahydrofolate synthetase 1; In the N-terminal section; belongs to the tetrahydrofolate dehydrogenase/cyclohydrolase family (935 aa) | |||
| MTFMT | Methionyl-tRNA formyltransferase, mitochondrial; Formylates methionyl-tRNA in mitochondria. A single tRNA(Met) gene gives rise to both an initiator and an elongator species via an unknown mechanism (By similarity) (389 aa) | |||
| ATIC | Bifunctional purine biosynthesis protein PURH; Bifunctional enzyme that catalyzes 2 steps in purine biosynthesis; Belongs to the PurH family (592 aa) | |||
| GOT2 | Aspartate aminotransferase, mitochondrial; Catalyzes the irreversible transamination of the L- tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids; Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family (430 aa) | |||
| ALDH1L2 | Mitochondrial 10-formyltetrahydrofolate dehydrogenase; Aldehyde dehydrogenase 1 family member L2; In the N-terminal section; belongs to the GART family (923 aa) | |||
| MTHFS | 5-formyltetrahydrofolate cyclo-ligase; Contributes to tetrahydrofolate metabolism. Helps regulate carbon flow through the folate-dependent one-carbon metabolic network that supplies carbon for the biosynthesis of purines, thymidine and amino acids. Catalyzes the irreversible conversion of 5-formyltetrahydrofolate (5-FTHF) to yield 5,10- methenyltetrahydrofolate (203 aa) | |||
| ASPA | Aspartoacylase; Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids (313 aa) | |||
| CAD | CAD protein; This protein is a "fusion" protein encoding four enzymatic activities of the pyrimidine pathway (GATase, CPSase, ATCase and DHOase); In the central section; belongs to the metallo- dependent hydrolases superfamily. DHOase family. CAD subfamily (2225 aa) | |||
| AMDHD1 | Probable imidazolonepropionase; Amidohydrolase domain containing 1 (426 aa) | |||
| ALDH1L1 | Cytosolic 10-formyltetrahydrofolate dehydrogenase; Aldehyde dehydrogenase 1 family member L1; In the N-terminal section; belongs to the GART family (912 aa) | |||
| AMT | Aminomethyltransferase, mitochondrial; The glycine cleavage system catalyzes the degradation of glycine (403 aa) | |||
| GLUD1 | Glutamate dehydrogenase 1, mitochondrial; Mitochondrial glutamate dehydrogenase that converts L- glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate (By similarity); Belongs to the Glu/Leu/Phe/Val dehydrogenases family (558 aa) | |||
| FTCD | Formimidoyltransferase-cyclodeaminase; Folate-dependent enzyme, that displays both transferase and deaminase activity. Serves to channel one-carbon units from formiminoglutamate to the folate pool; In the N-terminal section; belongs to the formiminotransferase family (541 aa) | |||
| GLUL | Glutamine synthetase; This enzyme has 2 functions- it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts; Belongs to the glutamine synthetase family (373 aa) | |||
| GLS2 | Glutaminase liver isoform, mitochondrial; Plays an important role in the regulation of glutamine catabolism. Promotes mitochondrial respiration and increases ATP generation in cells by catalyzing the synthesis of glutamate and alpha-ketoglutarate. Increases cellular anti-oxidant function via NADH and glutathione production. May play a role in preventing tumor proliferation; Ankyrin repeat domain containing (602 aa) | |||
| SHMT1 | Serine hydroxymethyltransferase, cytosolic; Interconversion of serine and glycine (483 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) | |||
| MTR | Methionine synthase; Catalyzes the transfer of a methyl group from methyl- cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofolate (By similarity); Belongs to the vitamin-B12 dependent methionine synthase family (1265 aa) | |||
| GART | Trifunctional purine biosynthetic protein adenosine-3; Phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase; In the central section; belongs to the AIR synthase family (1010 aa) | |||
| MTHFD2 | Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase, mitochondrial; Although its dehydrogenase activity is NAD-specific, it can also utilize NADP at a reduced efficiency; Belongs to the tetrahydrofolate dehydrogenase/cyclohydrolase family (350 aa) | |||
| DHFRL1 | Dihydrofolate reductase 2, mitochondrial; Key enzyme in folate metabolism. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. Required to prevent uracil accumulation in mtDNA. Binds its own mRNA and that of DHFR (187 aa) | |||
| MTHFD2L | Probable bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase 2; Methylenetetrahydrofolate dehydrogenase 2 like (347 aa) | |||
| DHFR | Dihydrofolate reductase; Key enzyme in folate metabolism. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. Catalyzes an essential reaction for de novo glycine and purine synthesis, and for DNA precursor synthesis. Binds its own mRNA and that of DHFR2 (187 aa) | |||
| ST20-MTHFS | 5-formyltetrahydrofolate cyclo-ligase; ST20-MTHFS readthrough; Belongs to the 5-formyltetrahydrofolate cyclo-ligase family (179 aa) | |||
| MTHFD1L | Monofunctional C1-tetrahydrofolate synthase, mitochondrial; May provide the missing metabolic reaction required to link the mitochondria and the cytoplasm in the mammalian model of one-carbon folate metabolism in embryonic an transformed cells complementing thus the enzymatic activities of MTHFD2; In the N-terminal section; belongs to the tetrahydrofolate dehydrogenase/cyclohydrolase family (979 aa) | |||
