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| GCLC | Glutamate-cysteine ligase catalytic subunit. (638 aa) | ||||
| PCK1 | Phosphoenolpyruvate carboxykinase, cytosolic [GTP]; Regulates cataplerosis and anaplerosis, the processes that control the levels of metabolic intermediates in the citric acid cycle. At low glucose levels, it catalyzes the cataplerotic 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. At high glucose levels, it catalyzes the anaplerotic conversion of phosphoenolpyruvate to oxaloacetate; Belongs to the phosphoenolpyruvate carboxykinase [...] (638 aa) | ||||
| GLS | Glutaminase. (603 aa) | ||||
| GAD2 | Glutamate decarboxylase 2. (585 aa) | ||||
| FPGS | Folylpolyglutamate synthase, mitochondrial; Catalyzes conversion of folates to polyglutamate derivatives allowing concentration of folate compounds in the cell and the intracellular retention of these cofactors, which are important substrates for most of the folate-dependent enzymes that are involved in one-carbon transfer reactions involved in purine, pyrimidine and amino acid synthesis; Belongs to the folylpolyglutamate synthase family. (585 aa) | ||||
| GLS2 | Glutaminase 2. (602 aa) | ||||
| MTRR | Methionine synthase reductase; Involved in the reductive regeneration of cob(I)alamin (vitamin B12) cofactor required for the maintenance of methionine synthase in a functional state. Necessary for utilization of methylgroups from the folate cycle, thereby affecting transgenerational epigenetic inheritance. Folate pathway donates methyl groups necessary for cellular methylation and affects different pathways such as DNA methylation, possibly explaining the transgenerational epigenetic inheritance effects. (695 aa) | ||||
| MDH2 | Malate dehydrogenase, mitochondrial. (338 aa) | ||||
| MTHFD1 | Methylenetetrahydrofolate dehydrogenase, cyclohydrolase and formyltetrahydrofolate synthetase 1. (935 aa) | ||||
| MRPS36 | 28S ribosomal protein S36, mitochondrial. (103 aa) | ||||
| ALDH18A1 | Delta-1-pyrroline-5-carboxylate synthase; In the C-terminal section; belongs to the gamma-glutamyl phosphate reductase family. (795 aa) | ||||
| GOT1 | Aspartate aminotransferase, cytoplasmic; Biosynthesis of L-glutamate from L-aspartate or L-cysteine. Important regulator of levels of glutamate, the major excitatory neurotransmitter of the vertebrate central nervous system. Acts as a scavenger of glutamate in brain neuroprotection. The aspartate aminotransferase activity is involved in hepatic glucose synthesis during development and in adipocyte glyceroneogenesis. Using L-cysteine as substrate, regulates levels of mercaptopyruvate, an important source of hydrogen sulfide. Mercaptopyruvate is converted into H(2)S via the action of 3-m [...] (413 aa) | ||||
| IDH2 | Isocitrate dehydrogenase [NADP], mitochondrial; Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (452 aa) | ||||
| SDHAF3 | Succinate dehydrogenase assembly factor 3, mitochondrial; Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF1. (125 aa) | ||||
| NAGS | N-acetylglutamate synthase. (534 aa) | ||||
| AMDHD1 | Probable imidazolonepropionase. (426 aa) | ||||
| GOT1L1 | Putative aspartate aminotransferase, cytoplasmic 2. (407 aa) | ||||
| SHMT1 | Serine hydroxymethyltransferase, cytosolic; Interconversion of serine and glycine. (484 aa) | ||||
| GCLM | Glutamate--cysteine ligase regulatory subunit; Belongs to the aldo/keto reductase family. Glutamate-- cysteine ligase light chain subfamily. (274 aa) | ||||
| IDH1 | Isocitrate dehydrogenase [NADP] cytoplasmic; May act as a corneal epithelial crystallin and may be involved in maintaining corneal epithelial transparency. (414 aa) | ||||
| FH | Fumarate hydratase. (510 aa) | ||||
| ALDH5A1 | Succinate-semialdehyde dehydrogenase; Belongs to the aldehyde dehydrogenase family. (526 aa) | ||||
| SHMT2 | Serine hydroxymethyltransferase, mitochondrial; Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis. Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate. 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. Also required for mitochondrial tran [...] (504 aa) | ||||
| ALDH1L1 | 10-formyltetrahydrofolate dehydrogenase; In the C-terminal section; belongs to the aldehyde dehydrogenase family. ALDH1L subfamily. (902 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. (975 aa) | ||||
| SDHA | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (Probable). Can act as a tumor suppressor (By similarity); Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily. (683 aa) | ||||
| PRODH | Proline dehydrogenase 1, mitochondrial; Converts proline to delta-1-pyrroline-5-carboxylate. (600 aa) | ||||
| AADAT | Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial; Transaminase with broad substrate specificity. Has transaminase activity towards aminoadipate, kynurenine, methionine and glutamate. Shows activity also towards tryptophan, aspartate and hydroxykynurenine. Accepts a variety of oxo-acids as amino-group acceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid, phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also use glyoxylate as amino-group acceptor (in vitro) (By similarity). (425 aa) | ||||
| PRODH2 | Hydroxyproline dehydrogenase; Dehydrogenase that converts trans-4-L-hydroxyproline to delta-1-pyrroline-3-hydroxy-5-carboxylate (Hyp) using ubiquinone-10 as the terminal electron acceptor. Can also use proline as a substrate but with a very much lower efficiency. Does not react with other diastereomers of Hyp: trans-4-D-hydroxyproline and cis-4-L- hydroxyproline. Ubiquininone analogs such as menadione, duroquinone and ubiquinone-1 react more efficiently than oxygen as the terminal electron acceptor during catalysis. (491 aa) | ||||
| ALDH4A1 | Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial; Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma- semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes (By similarity). (588 aa) | ||||
| ME2 | Malic enzyme. (607 aa) | ||||
| ACLY | ATP-citrate synthase; Catalyzes the cleavage of citrate into oxaloacetate and acetyl-CoA, the latter serving as common substrate for de novo cholesterol and fatty acid synthesis; In the N-terminal section; belongs to the succinate/malate CoA ligase beta subunit family. (1101 aa) | ||||
| ACSF3 | Malonate--CoA ligase ACSF3, mitochondrial; 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; Belongs to the ATP-dependent AMP-binding enzyme family. (630 aa) | ||||
| HOGA1 | 4-hydroxy-2-oxoglutarate aldolase, mitochondrial; Catalyzes the final step in the metabolic pathway of hydroxyproline; Belongs to the DapA family. (429 aa) | ||||
| UROC1 | Urocanate hydratase 1. (764 aa) | ||||
| KMO | Kynurenine 3-monooxygenase; Catalyzes the hydroxylation of L-kynurenine (L-Kyn) to form 3-hydroxy-L-kynurenine (L-3OHKyn). Required for synthesis of quinolinic acid, a neurotoxic NMDA receptor antagonist and potential endogenous inhibitor of NMDA receptor signaling in axonal targeting, synaptogenesis and apoptosis during brain development. Quinolinic acid may also affect NMDA receptor signaling in pancreatic beta cells, osteoblasts, myocardial cells, and the gastrointestinal tract. (471 aa) | ||||
| D2HGDH | D-2-hydroxyglutarate dehydrogenase, mitochondrial; Catalyzes the oxidation of D-2-hydroxyglutarate to alpha- ketoglutarate. (544 aa) | ||||
| QPRT | Nicotinate-nucleotide pyrophosphorylase [carboxylating]; Involved in the catabolism of quinolinic acid (QA). Belongs to the NadC/ModD family. (305 aa) | ||||
| HAL | Histidine ammonia-lyase. (692 aa) | ||||
| ACOT8 | Acyl-CoA thioesterase 8. (337 aa) | ||||
| KYNU | Kynureninase; Catalyzes the cleavage of L-kynurenine (L-Kyn) and L-3- hydroxykynurenine (L-3OHKyn) into anthranilic acid (AA) and 3- hydroxyanthranilic acid (3-OHAA), respectively. Has a preference for the L-3-hydroxy form. Also has cysteine-conjugate-beta-lyase activity. Belongs to the kynureninase family. (495 aa) | ||||
| GLS-2 | ANK_REP_REGION domain-containing protein. (93 aa) | ||||
| ME3 | Malic enzyme. (604 aa) | ||||
| KYAT3 | Kynurenine--oxoglutarate transaminase 3; Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). May catalyze the beta-elimination of S-conjugates and Se-conjugates of L- (seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond (By similarity). Has transaminase activity towards L-kynurenine, tryptophan, phenylalanine, serine, cysteine, methionine, histidine, glutamine and asparagine with glyoxylate as an amino group acceptor (in vitro). Has lower activity with 2-oxoglutarate as amino group acceptor (in vitro) (By sim [...] (455 aa) | ||||
| FOLR1 | Folate receptor alpha. (258 aa) | ||||
| GPT2 | Glutamic--pyruvic transaminase 2. (567 aa) | ||||
| ADHFE1 | Hydroxyacid-oxoacid transhydrogenase, mitochondrial; Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). L-3-hydroxybutyrate (L-3-OHB) is also a substrate for HOT when using 2- KG as hydrogen acceptor, resulting in the formation of D-2-HG (By similarity); Belongs to the iron-containing alcohol dehydrogenase family. Hydroxyacid-oxoacid transhydrogenase subfamily. (466 aa) | ||||
| HAAO | 3-hydroxyanthranilate 3,4-dioxygenase; Catalyzes the oxidative ring opening of 3-hydroxyanthranilate to 2-amino-3-carboxymuconate semialdehyde, which spontaneously cyclizes to quinolinate; Belongs to the 3-HAO family. (406 aa) | ||||
| NIT2 | Omega-amidase NIT2; Has a omega-amidase activity. The role of omega-amidase is to remove potentially toxic intermediates by converting alpha- ketoglutaramate and alpha-ketosuccinamate to biologically useful alpha- ketoglutarate and oxaloacetate, respectively. Belongs to the carbon-nitrogen hydrolase superfamily. NIT1/NIT2 family. (283 aa) | ||||
| ME1 | Malic enzyme. (571 aa) | ||||
| ALDH1L2 | 10-formyltetrahydrofolate dehydrogenase; In the C-terminal section; belongs to the aldehyde dehydrogenase family. ALDH1L subfamily. (923 aa) | ||||
| OAT | Ornithine aminotransferase, mitochondrial. (439 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 (By similarity). (430 aa) | ||||
| GAD1 | Glutamate decarboxylase 1; Catalyzes the production of GABA; Belongs to the group II decarboxylase family. (594 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; Belongs to the Glu/Leu/Phe/Val dehydrogenases family. (561 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 (By similarity). (187 aa) | ||||
| ASL | Argininosuccinate lyase; Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. (503 aa) | ||||
| DLST | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); Dihydrolipoamide succinyltransferase (E2) component of the 2- oxoglutarate dehydrogenase complex (By similarity). The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2- oxoglutarate to succinyl-CoA and CO(2) (By similarity). The 2- oxoglutarate dehydrogenase complex is mainly active in the mitochondrion. A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and p [...] (529 aa) | ||||
| OGDH | 2-oxoglutarate dehydrogenase, mitochondrial; 2-oxoglutarate dehydrogenase (E1) component of the 2- oxoglutarate dehydrogenase complex, which mediates the decarboxylation of alpha-ketoglutarate. The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion. A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl- Co [...] (1038 aa) | ||||
| ASS1 | Argininosuccinate synthase; One of the enzymes of the urea cycle, the metabolic pathway transforming neurotoxic amonia produced by protein catabolism into inocuous urea in the liver of ureotelic animals. Catalyzes the formation of arginosuccinate from aspartate, citrulline and ATP and together with ASL it is responsible for the biosynthesis of arginine in most body tissues; Belongs to the argininosuccinate synthase family. Type 1 subfamily. (426 aa) | ||||
| DDO | D-aspartate oxidase; Belongs to the DAMOX/DASOX family. (341 aa) | ||||
| FTCD | Formimidoyltransferase cyclodeaminase. (625 aa) | ||||
| TAT | Tyrosine aminotransferase; Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity for phenylalanine (By similarity). (454 aa) | ||||
| MDH1B | Putative malate dehydrogenase 1B; Belongs to the LDH/MDH superfamily. MDH type 2 family. (522 aa) | ||||
| MDH1 | Malate dehydrogenase, cytoplasmic. (334 aa) | ||||