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Gad2 Gad2 Mdh2 Mdh2 Shmt1 Shmt1 Adss Adss Amdhd1 Amdhd1 Acsf3 Acsf3 Sdhb Sdhb Ggt1 Ggt1 Cs Cs Prodh Prodh Tat Tat Ftcd Ftcd Haao Haao Aldh18a1 Aldh18a1 Lipf Lipf Me2 Me2 Nit2 Nit2 Ogdhl Ogdhl Glud1 Glud1 Dhfr Dhfr Sdha Sdha Adssl1 Adssl1 Acot4 Acot4 Mthfd1 Mthfd1 Aldh1l2 Aldh1l2 Ddo Ddo Mtrr Mtrr Idh3a Idh3a Uroc1 Uroc1 Asl Asl Sucla2 Sucla2 Hal Hal Slc25a12 Slc25a12 Adhfe1 Adhfe1 Aldh1l1 Aldh1l1 Mthfd1l Mthfd1l Gls Gls Mdh1b Mdh1b D2hgdh D2hgdh Dld Dld Mrps36 Mrps36 Acly Acly Idh2 Idh2 Folr1 Folr1 Kyat3 Kyat3 Mdh1 Mdh1 Ass1 Ass1 Acot8 Acot8 Idh1 Idh1 Gad1 Gad1 Ogdh Ogdh Glul Glul Oat Oat Hoga1 Hoga1 Htt Htt Aadat Aadat Mthfd2l Mthfd2l Dglucy Dglucy Suclg1 Suclg1 Pcx Pcx Prodh2 Prodh2 Dlst Dlst Idh3g Idh3g Aasdhppt Aasdhppt Csl Csl Nags Nags Acmsd Acmsd Gls2 Gls2 Grhpr Grhpr Aldh4a1 Aldh4a1 Got1l1 Got1l1 Sdhaf3 Sdhaf3 Aldh5a1 Aldh5a1 Kmo Kmo Pck2 Pck2 Me1 Me1 Gclc Gclc Gpt2 Gpt2 Got2 Got2 Qprt Qprt Me3 Me3 Gclm Gclm Pck1 Pck1 Idh3b Idh3b Kynu Kynu Fpgs Fpgs Got1 Got1 Shmt2 Shmt2 Atic Atic Fh1 Fh1 Phyh Phyh
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Gad2Glutamate decarboxylase 2; Catalyzes the production of GABA. (585 aa)
Mdh2Malate dehydrogenase, mitochondrial. (338 aa)
Shmt1Serine hydroxymethyltransferase, cytosolic; Interconversion of serine and glycine. (478 aa)
AdssAdenylosuccinate synthetase isozyme 2; Plays an important role in the de novo pathway and in the salvage pathway of purine nucleotide biosynthesis. Catalyzes the first committed step in the biosynthesis of AMP from IMP. Belongs to the adenylosuccinate synthetase family. (456 aa)
Amdhd1Probable imidazolonepropionase. (426 aa)
Acsf3Malonate--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. (583 aa)
SdhbSuccinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Iron-sulfur protein (IP) 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). (282 aa)
Ggt1Glutathione hydrolase 1 heavy chain; Cleaves the gamma-glutamyl bond of extracellular glutathione (gamma-Glu-Cys-Gly), glutathione conjugates, and other gamma-glutamyl compounds. The metabolism of glutathione releases free glutamate and the dipeptide cysteinyl-glycine, which is hydrolyzed to cysteine and glycine by dipeptidases. In the presence of high concentrations of dipeptides and some amino acids, can also catalyze a transpeptidation reaction, transferring the gamma-glutamyl moiety to an acceptor amino acid to form a new gamma-glutamyl compound. Initiates extracellular glutathione [...] (568 aa)
CsCitrate synthase, mitochondrial; Belongs to the citrate synthase family. (464 aa)
ProdhProline dehydrogenase 1, mitochondrial; Converts proline to delta-1-pyrroline-5-carboxylate. (599 aa)
TatTyrosine 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. Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. (454 aa)
FtcdFormimidoyltetrahydrofolate cyclodeaminase; Folate-dependent enzyme, that displays both transferase and deaminase activity. Serves to channel one-carbon units from formiminoglutamate to the folate pool (By similarity); In the N-terminal section; belongs to the formiminotransferase family. (541 aa)
Haao3-hydroxyanthranilate 3,4-dioxygenase; Catalyzes the oxidative ring opening of 3-hydroxyanthranilate to 2-amino-3-carboxymuconate semialdehyde, which spontaneously cyclizes to quinolinate. (286 aa)
Aldh18a1Delta-1-pyrroline-5-carboxylate synthase; Bifunctional enzyme that converts glutamate to glutamate 5- semialdehyde, an intermediate in the biosynthesis of proline, ornithine and arginine; In the C-terminal section; belongs to the gamma-glutamyl phosphate reductase family. (795 aa)
LipfGastric triacylglycerol lipase; Belongs to the AB hydrolase superfamily. Lipase family. (395 aa)
Me2NAD-dependent malic enzyme, mitochondrial. (589 aa)
Nit2Omega-amidase NIT2; Possesses omega-amidase activity. The role of omega-amidase is to remove potentially toxic intermediates by converting 2-oxoglutaramate and 2-oxosuccinamate to biologically useful 2-oxoglutarate and oxaloacetate, respectively. Can hydrolyze glutaramate, succinate and gamma- monomethyl-alpha-ketoglutarate in vitro. (276 aa)
OgdhlOxoglutarate dehydrogenase-like. (1029 aa)
Glud1Glutamate 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. (558 aa)
DhfrDihydrofolate 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. (187 aa)
SdhaSuccinate 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). Can act as a tumor suppressor. Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily. (664 aa)
Adssl1Adenylosuccinate synthetase isozyme 1; Component of the purine nucleotide cycle (PNC), which interconverts IMP and AMP to regulate the nucleotide levels in various tissues, and which contributes to glycolysis and ammoniagenesis. Catalyzes the first committed step in the biosynthesis of AMP from IMP. Belongs to the adenylosuccinate synthetase family. (457 aa)
Acot4Peroxisomal succinyl-coenzyme A thioesterase; Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH. In contrast to human peroxisomal succinyl-coenzyme A thioesterase/ACOT4, mouse ACOT4 is essentially a succinyl-CoA thioesterase with no activity with medium to long chain saturated acyl- CoAs and with a low activity toward glutaryl-CoA. Belongs to the C/M/P thioester hydrolase family. (421 aa)
Mthfd1C-1-tetrahydrofolate synthase, cytoplasmic, N-terminally processed; In the N-terminal section; belongs to the tetrahydrofolate dehydrogenase/cyclohydrolase family. (935 aa)
Aldh1l2Mitochondrial 10-formyltetrahydrofolate dehydrogenase; In the N-terminal section; belongs to the GART family. (923 aa)
DdoD-aspartate oxidase; Selectively catalyzes the oxidative deamination of D- aspartate and its N-methylated derivative, N-methyl D-aspartate. Belongs to the DAMOX/DASOX family. (341 aa)
MtrrMethionine 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. (696 aa)
Idh3aIsocitrate dehydrogenase [NAD] subunit alpha, mitochondrial; Catalytic subunit of the enzyme which catalyzes the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers. (366 aa)
Uroc1Urocanate hydratase. (710 aa)
AslArgininosuccinate lyase; Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. (464 aa)
Sucla2Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (463 aa)
HalHistidine ammonia-lyase; Belongs to the PAL/histidase family. (657 aa)
Slc25a12Calcium-binding mitochondrial carrier protein Aralar1; Mitochondrial and calcium-binding carrier that catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle. (677 aa)
Adhfe1Hydroxyacid-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). (465 aa)
Aldh1l1Cytosolic 10-formyltetrahydrofolate dehydrogenase; In the N-terminal section; belongs to the GART family. (902 aa)
Mthfd1lMonofunctional 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. (977 aa)
GlsGlutaminase kidney isoform, mitochondrial 65 kDa chain; Catalyzes the first reaction in the primary pathway for the renal catabolism of glutamine. Plays a role in maintaining acid-base homeostasis. Regulates the levels of the neurotransmitter glutamate, the main excitatory neurotransmitter in the brain. (674 aa)
Mdh1bPutative malate dehydrogenase 1B; Belongs to the LDH/MDH superfamily. MDH type 2 family. (500 aa)
D2hgdhD-2-hydroxyglutarate dehydrogenase, mitochondrial; Catalyzes the oxidation of D-2-hydroxyglutarate to alpha- ketoglutarate. (557 aa)
DldDihydrolipoyl dehydrogenase, mitochondrial; Lipoamide dehydrogenase is a component of the glycine cleavage system as well as an E3 component of three alpha-ketoacid dehydrogenase complexes (pyruvate-, alpha-ketoglutarate-, and branched- chain amino acid-dehydrogenase complex) (By similarity). The 2- oxoglutarate dehydrogenase complex is mainly active in the mitochondrion (By similarity). 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-CoA t [...] (509 aa)
Mrps3628S ribosomal protein S36, mitochondrial. (102 aa)
AclyATP-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 C-terminal section; belongs to the succinate/malate CoA ligase alpha subunit family. (1101 aa)
Idh2Isocitrate 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)
Folr1Folate receptor alpha; Binds to folate and reduced folic acid derivatives and mediates delivery of 5-methyltetrahydrofolate and folate analogs into the interior of cells. Has high affinity for folate and folic acid analogs at neutral pH. Exposure to slightly acidic pH after receptor endocytosis triggers a conformation change that strongly reduces its affinity for folates and mediates their release. Required for normal embryonic development and normal cell proliferation. Required for renal folate reabsorption. (255 aa)
Kyat3Kynurenine--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). (455 aa)
Mdh1Malate dehydrogenase, cytoplasmic. (334 aa)
Ass1Argininosuccinate 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. (412 aa)
Acot8Acyl-coenzyme A thioesterase 8; Acyl-coenzyme A (acyl-CoA) thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH. Acyl-coenzyme A thioesterase 8/ACOT8 display no strong substrate specificity with respect to the carboxylic acid moiety of Acyl-CoAs. Hydrolyzes medium length (C2 to C20) straight-chain, saturated and unsaturated acyl-CoAS but is inactive towards substrates with longer aliphatic chains. Moreover, it catalyz [...] (320 aa)
Idh1Isocitrate dehydrogenase [NADP] cytoplasmic; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (414 aa)
Gad1Glutamate decarboxylase 1; Catalyzes the production of GABA; Belongs to the group II decarboxylase family. (593 aa)
Ogdh2-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 [...] (1034 aa)
GlulGlutamine synthetase; Glutamine synthetase that catalyzes the ATP-dependent conversion of glutamate and ammonia to glutamine (By similarity). Its role depends on tissue localization: in the brain, it regulates the levels of toxic ammonia and converts neurotoxic glutamate to harmless glutamine, whereas in the liver, it is one of the enzymes responsible for the removal of ammonia. Essential for proliferation of fetal skin fibroblasts (By similarity). Independently of its glutamine synthetase activity, required for endothelial cell migration during vascular development. Involved in angiog [...] (373 aa)
OatOrnithine aminotransferase, mitochondrial. (439 aa)
Hoga14-hydroxy-2-oxoglutarate aldolase, mitochondrial; Catalyzes the final step in the metabolic pathway of hydroxyproline. (321 aa)
HttHuntingtin, myristoylated N-terminal fragment; [Huntingtin]: May play a role in microtubule-mediated transport or vesicle function. (3120 aa)
AadatKynurenine/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). Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. (425 aa)
Mthfd2lProbable bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase 2. (338 aa)
DglucyD-glutamate cyclase, mitochondrial; D-glutamate cyclase that converts D-glutamate to 5-oxo-D- proline. (617 aa)
Suclg1Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits. (346 aa)
PcxPyruvate carboxylase, mitochondrial; 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. (1179 aa)
Prodh2Hydroxyproline 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. (456 aa)
Dlst2-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 [...] (454 aa)
Idh3gIsocitrate dehydrogenase [NAD] subunit gamma 1, mitochondrial; Regulatory subunit which plays a role in the allosteric regulation of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers. (393 aa)
AasdhpptL-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase; Catalyzes the post-translational modification of target proteins by phosphopantetheine. Can transfer the 4'-phosphopantetheine moiety from coenzyme A to a serine residue of a broad range of acceptors, such as the acyl carrier domain of FASN (in vitro) (By similarity); Belongs to the P-Pant transferase superfamily. AcpS family. (309 aa)
CslCitrate synthase; Belongs to the citrate synthase family. (466 aa)
NagsN-acetylglutamate synthase conserved domain form; Plays a role in the regulation of ureagenesis by producing the essential cofactor N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPS1) activity. Belongs to the acetyltransferase family. (527 aa)
Acmsd2-amino-3-carboxymuconate-6-semialdehyde decarboxylase; Converts alpha-amino-beta-carboxymuconate-epsilon- semialdehyde (ACMS) to alpha-aminomuconate semialdehyde (AMS). ACMS can be converted non-enzymatically to quinolate (QA), a key precursor of NAD, and a potent endogenous excitotoxin of neuronal cells which is implicated in the pathogenesis of various neurodegenerative disorders. In the presence of ACMSD, ACMS is converted to AMS, a benign catabolite. ACMSD ultimately controls the metabolic fate of tryptophan catabolism along the kynurenine pathway; Belongs to the metallo-dependent [...] (336 aa)
Gls2Glutaminase 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. (602 aa)
GrhprGlyoxylate reductase/hydroxypyruvate reductase; Enzyme with hydroxy-pyruvate reductase, glyoxylate reductase and D-glycerate dehydrogenase enzymatic activities. Reduces hydroxypyruvate to D-glycerate, glyoxylate to glycolate oxidizes D- glycerate to hydroxypyruvate (By similarity). (328 aa)
Aldh4a1Delta-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). (562 aa)
Got1l1Putative aspartate aminotransferase, cytoplasmic 2; Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. (404 aa)
Sdhaf3Succinate 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)
Aldh5a1Succinate-semialdehyde dehydrogenase, mitochondrial; Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA); Belongs to the aldehyde dehydrogenase family. (523 aa)
KmoKynurenine 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. (479 aa)
Pck2Phosphoenolpyruvate 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. (667 aa)
Me1NADP-dependent malic enzyme. (572 aa)
GclcGlutamate--cysteine ligase catalytic subunit; Belongs to the glutamate--cysteine ligase type 3 family. (637 aa)
Gpt2Alanine aminotransferase 2; Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. (522 aa)
Got2Aspartate 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. (430 aa)
QprtNicotinate-nucleotide pyrophosphorylase [carboxylating]; Involved in the catabolism of quinolinic acid (QA). Belongs to the NadC/ModD family. (299 aa)
Me3NADP-dependent malic enzyme, mitochondrial; Belongs to the malic enzymes family. (604 aa)
GclmGlutamate--cysteine ligase regulatory subunit; Belongs to the aldo/keto reductase family. Glutamate-- cysteine ligase light chain subfamily. (274 aa)
Pck1Phosphoenolpyruvate 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 [...] (622 aa)
Idh3bIsocitrate dehydrogenase [NAD] subunit, mitochondrial. (384 aa)
KynuKynureninase; 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. (465 aa)
FpgsFolylpolyglutamate 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. Dihydrofolate, tetrahydrofolate, 5,10- methylenetetrahydrofolate, 10-formyltetrahydrofolate and 5- formyltetrahydrofolate are the best substrates. Folic acid and 5- methyltetrahydrofolate can also act [...] (587 aa)
Got1Aspartate 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)
Shmt2Serine hydroxymethyltransferase, mitochondrial; Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis (By similarity). Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate (By similarity). 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 (By s [...] (504 aa)
AticPhosphoribosylaminoimidazolecarboxamide formyltransferase; Bifunctional enzyme that catalyzes 2 steps in purine biosynthesis; Belongs to the PurH family. (592 aa)
Fh1Fumarate hydratase, mitochondrial; Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. Experiments in different species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable). [Isoform Cytoplasmic]: Catalyzes the dehydration of L-malate to fumarate. Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by- product of the urea cycle and amino-acid catabolism. Also plays a role in DNA repair by promoting non-homologous end-joining [...] (507 aa)
PhyhPhytanoyl-CoA dioxygenase, peroxisomal; Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. (338 aa)
Your Current Organism:
Mus musculus
NCBI taxonomy Id: 10090
Other names: LK3 transgenic mice, M. musculus, Mus sp. 129SV, house mouse, mouse, nude mice, transgenic mice
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