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ACO2 | Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis- aconitate; Belongs to the aconitase/IPM isomerase family. (780 aa) | ||||
TPI1 | Triosephosphate isomerase; Triosephosphate isomerase is an extremely efficient metabolic enzyme that catalyzes the interconversion between dihydroxyacetone phosphate (DHAP) and D-glyceraldehyde-3-phosphate (G3P) in glycolysis and gluconeogenesis. (285 aa) | ||||
ALDH6A1 | Methylmalonate-semialdehyde dehydrogenase acylating, mitochondrial isoform 1. (534 aa) | ||||
GPI | Glucose-6-phosphate isomerase; In the cytoplasm, catalyzes the conversion of glucose-6- phosphate to fructose-6-phosphate, the second step in glycolysis, and the reverse reaction during gluconeogenesis (By similarity). Besides it's role as a glycolytic enzyme, also acts as a secreted cytokine: acts as an angiogenic factor (AMF) that stimulates endothelial cell motility. Acts as a neurotrophic factor, neuroleukin, for spinal and sensory neurons. It is secreted by lectin-stimulated T-cells and induces immunoglobulin secretion (By similarity). (558 aa) | ||||
GLDC | Glycine cleavage system P protein; The glycine cleavage system catalyzes the degradation of glycine. (1027 aa) | ||||
RPIA | Ribose-5-phosphate isomerase; Belongs to the ribose 5-phosphate isomerase family. (306 aa) | ||||
HK2 | Hexokinase-2; Catalyzes the phosphorylation of hexose, such as D-glucose and D-fructose, to hexose 6-phosphate (D-glucose 6-phosphate and D- fructose 6-phosphate, respectively). Mediates the initial step of glycolysis by catalyzing phosphorylation of D-glucose to D-glucose 6- phosphate. Plays a key role in maintaining the integrity of the outer mitochondrial membrane by preventing the release of apoptogenic molecules from the intermembrane space and subsequent apoptosis. Belongs to the hexokinase family. (917 aa) | ||||
HADHA | Trifunctional enzyme subunit alpha, mitochondrial; Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional en [...] (763 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). (558 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) | ||||
ENO4 | Enolase 4. (617 aa) | ||||
GLYCTK | Glycerate kinase. (523 aa) | ||||
PGK1 | Phosphoglycerate kinase 1; Catalyzes one of the two ATP producing reactions in the glycolytic pathway via the reversible conversion of 1,3- diphosphoglycerate to 3-phosphoglycerate. In addition to its role as a glycolytic enzyme, it seems that PGK-1 acts as a polymerase alpha cofactor protein (primer recognition protein). May play a role in sperm motility. (417 aa) | ||||
PRPS1 | Uncharacterized protein. (319 aa) | ||||
TKFC | ATP-dependent dihydroxyacetone kinase; Catalyzes both the phosphorylation of dihydroxyacetone and of glyceraldehyde, and the splitting of ribonucleoside diphosphate-X compounds among which FAD is the best substrate. Represses IFIH1-mediated cellular antiviral response (By similarity). Belongs to the dihydroxyacetone kinase (DAK) family. (508 aa) | ||||
CAT | Catalase; Occurs in almost all aerobically respiring organisms and serves to protect cells from the toxic effects of hydrogen peroxide. Promotes growth of cells; Belongs to the catalase family. (550 aa) | ||||
ME3 | Malic enzyme. (604 aa) | ||||
SDHD | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Membrane-anchoring 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); Belongs to the CybS family. (177 aa) | ||||
AGXT | Serine--pyruvate aminotransferase. (402 aa) | ||||
PGAM2 | Phosphoglycerate mutase; Belongs to the phosphoglycerate mutase family. BPG- dependent PGAM subfamily. (253 aa) | ||||
OGDH | Oxoglutarate dehydrogenase. (1035 aa) | ||||
TKTL1 | Transketolase-like protein 1. (596 aa) | ||||
ENO2 | Enolase 2. (434 aa) | ||||
TKTL2 | Transketolase like 2. (589 aa) | ||||
IDH1 | Isocitrate dehydrogenase [NADP]; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (463 aa) | ||||
RGN | Regucalcin; Gluconolactonase with low activity towards other sugar lactones, including gulonolactone and galactonolactone. Catalyzes a key step in ascorbic acid (vitamin C) biosynthesis. Can also hydrolyze diisopropyl phosphorofluoridate and phenylacetate (in vitro). Calcium- binding protein. Modulates Ca(2+) signaling, and Ca(2+)-dependent cellular processes and enzyme activities (By similarity). Belongs to the SMP-30/CGR1 family. (342 aa) | ||||
AMT | Aminomethyltransferase; The glycine cleavage system catalyzes the degradation of glycine; Belongs to the GcvT family. (403 aa) | ||||
EHHADH | Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase; Belongs to the enoyl-CoA hydratase/isomerase family. (731 aa) | ||||
ACAT1 | Acetyl-CoA acetyltransferase 1; Belongs to the thiolase-like superfamily. Thiolase family. (426 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. (664 aa) | ||||
HAO1 | Hydroxyacid oxidase 1. (370 aa) | ||||
PDHA1 | Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle. (396 aa) | ||||
PFKM | ATP-dependent 6-phosphofructokinase, muscle type; Catalyzes the phosphorylation of D-fructose 6-phosphate to fructose 1,6-bisphosphate by ATP, the first committing step of glycolysis; Belongs to the phosphofructokinase type A (PFKA) family. ATP-dependent PFK group I subfamily. Eukaryotic two domain clade 'E' sub-subfamily. (810 aa) | ||||
PSPH | Phosphoserine phosphatase isoform X1. (225 aa) | ||||
ME2 | Malic enzyme. (678 aa) | ||||
HKDC1 | Hexokinase domain containing 1. (917 aa) | ||||
RPE | Ribulose-phosphate 3-epimerase; Belongs to the ribulose-phosphate 3-epimerase family. (228 aa) | ||||
ACOX1 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (660 aa) | ||||
SHMT1 | Serine hydroxymethyltransferase; Interconversion of serine and glycine. Belongs to the SHMT family. (646 aa) | ||||
ENO3 | Beta-enolase; Appears to have a function in striated muscle development and regeneration; Belongs to the enolase family. (476 aa) | ||||
GAPDH | Glyceraldehyde-3-phosphate dehydrogenase; Has both glyceraldehyde-3-phosphate dehydrogenase and nitrosylase activities, thereby playing a role in glycolysis and nuclear functions, respectively. Glyceraldehyde-3-phosphate dehydrogenase is a key enzyme in glycolysis that catalyzes the first step of the pathway by converting D-glyceraldehyde 3-phosphate (G3P) into 3-phospho-D-glyceroyl phosphate. Modulates the organization and assembly of the cytoskeleton. Facilitates the CHP1-dependent microtubule and membrane associations through its ability to stimulate the binding of CHP1 to microtubu [...] (398 aa) | ||||
MCEE | Methylmalonyl-CoA epimerase. (173 aa) | ||||
OGDHL | Oxoglutarate dehydrogenase like. (978 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. (452 aa) | ||||
PKLR | Pyruvate kinase; Belongs to the pyruvate kinase family. (565 aa) | ||||
IDNK | Gluconokinase; Belongs to the gluconokinase GntK/GntV family. (193 aa) | ||||
ACADS | Short-chain specific acyl-CoA dehydrogenase, mitochondrial; Short-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, short-chai [...] (612 aa) | ||||
ECHS1 | Enoyl-CoA hydratase, mitochondrial; Belongs to the enoyl-CoA hydratase/isomerase family. (328 aa) | ||||
PFKL | 6-phosphofructokinase; Belongs to the phosphofructokinase type A (PFKA) family. ATP-dependent PFK group I subfamily. Eukaryotic two domain clade "E" sub-subfamily. (782 aa) | ||||
GOT1L1 | Glutamic-oxaloacetic transaminase 1 like 1. (404 aa) | ||||
PGAM1 | Phosphoglycerate mutase; Belongs to the phosphoglycerate mutase family. BPG- dependent PGAM subfamily. (254 aa) | ||||
PGK2 | Phosphoglycerate kinase 2; Essential for sperm motility and male fertility but is not required for the completion of spermatogenesis. Belongs to the phosphoglycerate kinase family. (417 aa) | ||||
FH | Fumarate 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 (By similarity). Also plays a role in DNA repair by promoting non-homolog [...] (466 aa) | ||||
SUCLA2 | Succinate--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) | ||||
ACSS1 | Acetyl-coenzyme A synthetase. (703 aa) | ||||
ENO1 | Alpha-enolase isoform X1. (434 aa) | ||||
ACAT2 | Acetyl-CoA acetyltransferase 2; Belongs to the thiolase-like superfamily. Thiolase family. (447 aa) | ||||
GCK | Glucokinase. (659 aa) | ||||
FBP2 | FBPase domain-containing protein; Belongs to the FBPase class 1 family. (344 aa) | ||||
ADH5 | S-(hydroxymethyl)glutathione dehydrogenase; Belongs to the zinc-containing alcohol dehydrogenase family. Class-III subfamily. (392 aa) | ||||
FBP1 | Fructose-1,6-bisphosphatase 1; Catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate in the presence of divalent cations, acting as a rate-limiting enzyme in gluconeogenesis. Plays a role in regulating glucose sensing and insulin secretion of pancreatic beta-cells. Appears to modulate glycerol gluconeogenesis in liver. Important regulator of appetite and adiposity; increased expression of the protein in liver after nutrient excess increases circulating satiety hormones and reduces appetite-stimulating neuropeptides and thus seems to provide a feedback mechanism [...] (338 aa) | ||||
ESD | S-formylglutathione hydrolase; Serine hydrolase involved in the detoxification of formaldehyde. (282 aa) | ||||
CPS1 | Carbamoyl-phosphate synthase 1. (1425 aa) | ||||
PCCA | Propionyl-CoA carboxylase alpha chain, mitochondrial; This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domai [...] (730 aa) | ||||
MTHFR | Methylenetetrahydrofolate reductase; Belongs to the methylenetetrahydrofolate reductase family. (700 aa) | ||||
PKM | Multifunctional fusion protein; Belongs to the pyruvate kinase family. (1202 aa) | ||||
ALDOC | Fructose-bisphosphate aldolase; Belongs to the class I fructose-bisphosphate aldolase family. (434 aa) | ||||
MMUT | Methylmalonyl-CoA mutase, mitochondrial; Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle; Belongs to the methylmalonyl-CoA mutase family. (750 aa) | ||||
GCSH | Glycine cleavage system H protein; The H protein shuttles the methylamine group of glycine from the P protein to the T protein; Belongs to the GcvH family. (173 aa) | ||||
PGP | Phosphoglycolate phosphatase; Belongs to the HAD-like hydrolase superfamily. (321 aa) | ||||
ACSS2 | Acetyl-coenzyme A synthetase, cytoplasmic isoform X1. (714 aa) | ||||
ALDOA | Fructose-bisphosphate aldolase; Belongs to the class I fructose-bisphosphate aldolase family. (532 aa) | ||||
PRPS1L1 | Pribosyltran_N domain-containing protein. (318 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). (442 aa) | ||||
CS | Citrate synthase, mitochondrial; Belongs to the citrate synthase family. (464 aa) | ||||
SHMT2 | Serine hydroxymethyltransferase; Interconversion of serine and glycine. Belongs to the SHMT family. (503 aa) | ||||
GPT | Alanine aminotransferase 1; Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity). (555 aa) | ||||
TALDO1 | Transaldolase; Transaldolase is important for the balance of metabolites in the pentose-phosphate pathway. (392 aa) | ||||
ACO1 | Aconitase 1; Belongs to the aconitase/IPM isomerase family. (889 aa) | ||||
HAO2 | Hydroxyacid oxidase 2 isoform 1. (353 aa) | ||||
IDH3B | Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial; Plays a structural role to facilitate the assembly and ensure the full activity 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. (602 aa) | ||||
DLD | Dihydrolipoyl 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 [...] (511 aa) | ||||
GPT2 | Glutamic--pyruvic transaminase 2. (547 aa) | ||||
IDH3G | Isocitrate dehydrogenase [NAD] subunit gamma, 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. (388 aa) | ||||
ADPGK | ADP dependent glucokinase. (497 aa) | ||||
MDH1 | Malate dehydrogenase, cytoplasmic. (352 aa) | ||||
HIBCH | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite; Belongs to the enoyl-CoA hydratase/isomerase family. (386 aa) | ||||
ACOX3 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (720 aa) | ||||
PHGDH | D-3-phosphoglycerate dehydrogenase; Catalyzes the reversible oxidation of 3-phospho-D-glycerate to 3-phosphonooxypyruvate, the first step of the phosphorylated L- serine biosynthesis pathway. Also catalyzes the reversible oxidation of 2-hydroxyglutarate to 2-oxoglutarate and the reversible oxidation of (S)-malate to oxaloacetate; Belongs to the D-isomer specific 2-hydroxyacid dehydrogenase family. (547 aa) | ||||
PCCB | Propionyl-CoA carboxylase beta chain, mitochondrial; This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain [...] (558 aa) | ||||
DLST | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); Dihydrolipoamide succinyltransferase (E2) component of the 2- oxoglutarate dehydrogenase complex. 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 (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 [...] (446 aa) | ||||
H6PD | Hexose-6-phosphate dehydrogenase/glucose 1-dehydrogenase. (812 aa) | ||||
PDHB | Pyruvate dehydrogenase E1 component subunit beta; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. (361 aa) | ||||
TKT | Transketolase. (598 aa) | ||||
PGD | 6-phosphogluconate dehydrogenase, decarboxylating; Catalyzes the oxidative decarboxylation of 6-phosphogluconate to ribulose 5-phosphate and CO(2), with concomitant reduction of NADP to NADPH. (482 aa) | ||||
MDH2 | Malate dehydrogenase, mitochondrial. (395 aa) | ||||
ALDOB | Fructose-bisphosphate aldolase; Belongs to the class I fructose-bisphosphate aldolase family. (366 aa) | ||||
HK1 | Uncharacterized protein. (919 aa) | ||||
DLAT | Acetyltransferase component of pyruvate dehydrogenase complex; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). (667 aa) | ||||
SDHB | Succinate 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). (328 aa) | ||||
ME1 | NADP-dependent malic enzyme; Belongs to the malic enzymes family. (571 aa) | ||||
SDHC | Succinate dehydrogenase cytochrome b560 subunit, mitochondrial; Membrane-anchoring 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); Belongs to the cytochrome b560 family. (176 aa) |