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| | NOS2 | Nitric oxide synthase, inducible; Produces nitric oxide (NO) which is a messenger molecule with diverse functions throughout the body. In macrophages, NO mediates tumoricidal and bactericidal actions. Also has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such PTGS2/COX2. As component of the iNOS-S100A8/9 transnitrosylase complex involved in the selective inflammatory stimulus-dependent S- nitrosylation of GAPDH implicated in regulation of the GAIT complex activity and probably multiple targets including ANXA5, EZR, MSN and VIM. Involved in i [...] (1156 aa) |
| | GCSH | Glycine cleavage system H protein, mitochondrial; The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST). Belongs to the GcvH family. (173 aa) |
| | LDHD | Lactate dehydrogenase D. (509 aa) |
| | ALDH1L2 | 10-formyltetrahydrofolate dehydrogenase; In the C-terminal section; belongs to the aldehyde dehydrogenase family. ALDH1L subfamily. (923 aa) |
| | DBT | 2-oxoisovalerate dehydrogenase E2 component (dihydrolipoyl transacylase); 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). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component. (482 aa) |
| | SLC25A17 | Solute carrier family 25 (Mitochondrial carrier; peroxisomal membrane protein, 34kDa), member 17; Belongs to the mitochondrial carrier (TC 2.A.29) family. (307 aa) |
| | AGXT | Serine--pyruvate aminotransferase. (414 aa) |
| | IL4I1 | Interleukin 4 induced 1. (578 aa) |
| | IDO2 | Indoleamine 2,3-dioxygenase 2. (367 aa) |
| | PEX13 | Peroxisomal membrane protein PEX13; Component of the peroxisomal translocation machinery with PEX14 and PEX17. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PAS10/PEX5). Involved in the import of PTS1 and PTS2 proteins (By similarity). (403 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) |
| | 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) |
| | IVD | Isovaleryl-CoA dehydrogenase, mitochondrial. (467 aa) |
| | ACOX2 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (682 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 (By similarity); Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (500 aa) |
| | PEX2 | Peroxisomal biogenesis factor 2. (305 aa) |
| | SCP2 | Non-specific lipid-transfer protein; Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis. (543 aa) |
| | HACL1 | 2-hydroxyacyl-CoA lyase 1; Belongs to the TPP enzyme family. (581 aa) |
| | AIG1 | Androgen induced 1. (238 aa) |
| | ACADVL | Very long-chain specific acyl-CoA dehydrogenase, mitochondrial; Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, myristoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons; Belongs to the acyl-CoA dehydrogenase family. (655 aa) |
| | GPT2 | Glutamic--pyruvic transaminase 2. (567 aa) |
| | ECHDC2 | Enoyl-CoA hydratase domain-containing protein 2, mitochondrial. (346 aa) |
| | ACAT2 | Acetyl-Coenzyme A acetyltransferase 2; Belongs to the thiolase-like superfamily. Thiolase family. (397 aa) |
| | BDH2 | 3-hydroxybutyrate dehydrogenase type 2; Dehydrogenase that mediates the formation of 2,5- dihydroxybenzoic acid (2,5-DHBA), a siderophore that shares structural similarities with bacterial enterobactin and associates with LCN2, thereby playing a key role in iron assimilation and homeostasis. Plays a role in susceptibility to bacterial infection by providing an assimilable source of iron that is exploited by pathogenic bacteria (By similarity). Also acts as a 3-hydroxybutyrate dehydrogenase (By similarity). (245 aa) |
| | AMT | Aminomethyltransferase, mitochondrial; The glycine cleavage system catalyzes the degradation of glycine; Belongs to the GcvT family. (397 aa) |
| | HADH | Hydroxyacyl-CoA dehydrogenase. (314 aa) |
| | OTC | Ornithine carbamoyltransferase, mitochondrial; Belongs to the aspartate/ornithine carbamoyltransferase superfamily. OTCase family. (354 aa) |
| | HIBADH | 3-hydroxyisobutyrate dehydrogenase, mitochondrial; Belongs to the HIBADH-related family. 3-hydroxyisobutyrate dehydrogenase subfamily. (336 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) |
| | AKR1A1 | Aldo-keto reductase family 1 member A1; Catalyzes the NADPH-dependent reduction of a wide variety of carbonyl-containing compounds to their corresponding alcohols. Displays enzymatic activity towards endogenous metabolites such as aromatic and aliphatic aldehydes, ketones, monosaccharides and bile acids, with a preference for negatively charged substrates, such as glucuronate and succinic semialdehyde (By similarity). Plays an important role in ascorbic acid biosynthesis by catalyzing the reduction of D-glucuronic acid and D-glucurono-gamma-lactone (By similarity). Functions as a detox [...] (325 aa) |
| | GNPDA2 | Glucosamine-6-phosphate isomerase 2. (341 aa) |
| | SDS | L-serine dehydratase/L-threonine deaminase; Belongs to the serine/threonine dehydratase family. (446 aa) |
| | NUDT19 | Nudix hydrolase 19. (381 aa) |
| | PON3 | Paraoxonase 3. (354 aa) |
| | DDAH1 | N(G),N(G)-dimethylarginine dimethylaminohydrolase 1; Hydrolyzes N(G),N(G)-dimethyl-L-arginine (ADMA) and N(G)- monomethyl-L-arginine (MMA) which act as inhibitors of NOS. Has therefore a role in the regulation of nitric oxide generation. (285 aa) |
| | AFMID | Kynurenine formamidase; Catalyzes the hydrolysis of N-formyl-L-kynurenine to L- kynurenine, the second step in the kynurenine pathway of tryptophan degradation. Kynurenine may be further oxidized to nicotinic acid, NAD(H) and NADP(H). Required for elimination of toxic metabolites. (315 aa) |
| | E1BEA7_BOVIN | 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) |
| | LOC513210 | Uncharacterized protein. (464 aa) |
| | QDPR | Dihydropteridine reductase; The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (242 aa) |
| | ACAD11 | Acyl-Coenzyme A dehydrogenase family, member 11. (781 aa) |
| | ABCD2 | ATP binding cassette subfamily D member 2. (741 aa) |
| | CYP26C1 | Cytochrome P450 family 26 subfamily C member 1; Belongs to the cytochrome P450 family. (523 aa) |
| | E1BBV5_BOVIN | 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. (166 aa) |
| | EHHADH | Enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase. (723 aa) |
| | HAO1 | Hydroxyacid oxidase 1. (387 aa) |
| | ARG2 | Arginase-2, mitochondrial; May play a role in the regulation of extra-urea cycle arginine metabolism and also in down-regulation of nitric oxide synthesis. Extrahepatic arginase functions to regulate L-arginine bioavailability to nitric oxid synthase (NOS). Arginine metabolism is a critical regulator of innate and adaptive immune responses. Seems to be involved in negative regulation of the survival capacity of activated T cells. May suppress inflammation-related signaling in asthmatic airway epithelium. May play a role in promoting prenatal immune suppression. Regulates RPS6KB1 signal [...] (462 aa) |
| | RENBP | N-acylglucosamine 2-epimerase; Catalyzes the interconversion of N-acetylglucosamine to N- acetylmannosamine. Binds to renin forming a protein complex called high molecular weight (HMW) renin and inhibits renin activity. Involved in the N-glycolylneuraminic acid (Neu5Gc) degradation pathway (By similarity); Belongs to the N-acylglucosamine 2-epimerase family. (432 aa) |
| | LOC100300115 | Uncharacterized protein. (333 aa) |
| | PRODH | Proline dehydrogenase 1, mitochondrial; Converts proline to delta-1-pyrroline-5-carboxylate. (600 aa) |
| | ENOSF1 | Mitochondrial enolase superfamily member 1; Plays a role in the catabolism of L-fucose, a sugar that is part of the carbohydrates that are attached to cellular glycoproteins. Catalyzes the dehydration of L-fuconate to 2-keto-3-deoxy-L-fuconate by the abstraction of the 2-proton to generate an enediolate intermediate that is stabilized by the magnesium ion. May down-regulate thymidylate synthase activity, possibly already at the RNA level, by promoting the degradation of TYMS mRNA via an antisense RNA-based mechanism. (443 aa) |
| | ECHDC1 | Ethylmalonyl-CoA decarboxylase; Decarboxylates ethylmalonyl-CoA, a potentially toxic metabolite, to form butyryl-CoA, suggesting it might be involved in metabolite proofreading. Also has methylmalonyl-CoA decarboxylase activity at lower level; Belongs to the enoyl-CoA hydratase/isomerase family. (306 aa) |
| | IDO1 | Indoleamine 2,3-dioxygenase 1. (454 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) |
| | ETFDH | Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; Accepts electrons from ETF and reduces ubiquinone. Belongs to the ETF-QO/FixC family. (873 aa) |
| | HGD | Homogentisate 1,2-dioxygenase. (447 aa) |
| | PPARD | Peroxisome proliferator activated receptor delta. (441 aa) |
| | CPT1A | Carnitine palmitoyltransferase 1A; Belongs to the carnitine/choline acetyltransferase family. (773 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) |
| | 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 (By similarity). Among the different mitochondrial acyl-CoA dehydrogen [...] (438 aa) |
| | ALDH6A1 | Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA. (537 aa) |
| | ASRGL1 | Isoaspartyl peptidase/L-asparaginase alpha chain; Has both L-asparaginase and beta-aspartyl peptidase activity. May be involved in the production of L-aspartate, which can act as an excitatory neurotransmitter in some brain regions. Is highly active with L-Asp beta-methyl ester. Besides, has catalytic activity toward beta-aspartyl dipeptides and their methyl esters, including beta-L-Asp- L-Phe, beta-L-Asp-L-Phe methyl ester (aspartame), beta-L-Asp-L-Ala, beta-L-Asp-L-Leu and beta-L-Asp-L-Lys. Does not have aspartylglucosaminidase activity and is inactive toward GlcNAc-L-Asn. Likewise, [...] (308 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) |
| | GCDH | Glutaryl-CoA dehydrogenase, mitochondrial; Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L- hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor (By similarity). (458 aa) |
| | DECR2 | 2,4-dienoyl-CoA reductase 2. (291 aa) |
| | ACOXL | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (631 aa) |
| | ECH1 | Enoyl-CoA hydratase 1; Belongs to the enoyl-CoA hydratase/isomerase family. (326 aa) |
| | LPIN1 | Lipin 1. (935 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) |
| | CRAT | Carnitine O-acetyltransferase; Belongs to the carnitine/choline acetyltransferase family. (626 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) |
| | HIBCH | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA (By similarity); Belongs to the enoyl-CoA hydratase/isomerase family. (389 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) |
| | QPRT | Nicotinate-nucleotide pyrophosphorylase [carboxylating]; Involved in the catabolism of quinolinic acid (QA). Belongs to the NadC/ModD family. (305 aa) |
| | ENSBTAP00000063081 | Uncharacterized protein. (313 aa) |
| | SLC27A2 | Solute carrier family 27 member 2. (633 aa) |
| | NOS3 | Nitric oxide synthase, endothelial; Produces nitric oxide (NO) which is implicated in vascular smooth muscle relaxation through a cGMP-mediated signal transduction pathway. NO mediates vascular endothelial growth factor (VEGF)-induced angiogenesis in coronary vessels and promotes blood clotting through the activation of platelets; Belongs to the NOS family. (1205 aa) |
| | LOC513508 | Uncharacterized protein. (847 aa) |
| | CBS | Cystathionine beta-synthase; Belongs to the cysteine synthase/cystathionine beta- synthase family. (571 aa) |
| | HPD | 4-hydroxyphenylpyruvate dioxygenase; Key enzyme in the degradation of tyrosine. (397 aa) |
| | AMDHD2 | N-acetylglucosamine-6-phosphate deacetylase; Hydrolyzes the N-glycolyl group from N-glycolylglucosamine 6- phosphate (GlcNGc-6-P) in the N-glycolylneuraminic acid (Neu5Gc) degradation pathway; Belongs to the metallo-dependent hydrolases superfamily. NagA family. (499 aa) |
| | ENSBTAP00000064241 | Amidase domain-containing protein. (364 aa) |
| | LPIN2 | Lipin 2. (952 aa) |
| | IDNK | Gluconokinase; Belongs to the gluconokinase GntK/GntV family. (233 aa) |
| | MCCC2 | Methylcrotonoyl-CoA carboxylase 2. (567 aa) |
| | HAL | Histidine ammonia-lyase. (692 aa) |
| | ADIPOQ | Adiponectin; Important adipokine involved in the control of fat metabolism and insulin sensitivity, with direct anti-diabetic, anti-atherogenic and anti-inflammatory activities. Stimulates AMPK phosphorylation and activation in the liver and the skeletal muscle, enhancing glucose utilization and fatty-acid combustion. Antagonizes TNF-alpha by negatively regulating its expression in various tissues such as liver and macrophages, and also by counteracting its effects. Inhibits endothelial NF-kappa-B signaling through a cAMP-dependent pathway. May play a role in cell growth, angiogenesis [...] (328 aa) |
| | GCAT | 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. (479 aa) |
| | NOS1 | Nitric oxide synthase; Produces nitric oxide (NO). (1359 aa) |
| | PAH | Phenylalanine-4-hydroxylase; Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine. (451 aa) |
| | ACOT8 | Acyl-CoA thioesterase 8. (337 aa) |
| | CPT1C | Carnitine palmitoyltransferase 1C. (811 aa) |
| | TDO2 | Tryptophan 2,3-dioxygenase; Heme-dependent dioxygenase that catalyzes the oxidative cleavage of the L-tryptophan (L-Trp) pyrrole ring and converts L- tryptophan to N-formyl-L-kynurenine. Catalyzes the oxidative cleavage of the indole moiety. (418 aa) |
| | AUH | Uncharacterized protein. (275 aa) |
| | BCKDK | [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex. (412 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) |
| | ACAD10 | Uncharacterized protein. (1098 aa) |
| | ENSBTAP00000067997 | Uncharacterized protein; Belongs to the peptidase C1 family. (112 aa) |
| | GLS-2 | ANK_REP_REGION domain-containing protein. (93 aa) |
| | ENSBTAP00000068994 | Uncharacterized protein. (193 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) |
| | FAAH | Fatty acid amide hydrolase. (608 aa) |
| | HADHA | Hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha; Belongs to the enoyl-CoA hydratase/isomerase family. (771 aa) |
| | GSTZ1 | Glutathione S-transferase zeta 1; Belongs to the GST superfamily. (445 aa) |
| | DPEP1 | Dipeptidase 1; Hydrolyzes a wide range of dipeptides. Implicated in the renal metabolism of glutathione and its conjugates. Converts leukotriene D4 to leukotriene E4; it may play an important role in the regulation of leukotriene activity (By similarity); Belongs to the metallo-dependent hydrolases superfamily. Peptidase M19 family. (477 aa) |
| | ACAA2 | 3-ketoacyl-CoA thiolase, mitochondrial; Belongs to the thiolase-like superfamily. Thiolase family. (455 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) |
| | ENSBTAP00000070244 | Uncharacterized protein; Belongs to the thiolase-like superfamily. Thiolase family. (372 aa) |
| | ACOX1 | Peroxisomal acyl-CoA oxidase 1, A chain; Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl- CoAs. (660 aa) |
| | HADHB | Trifunctional enzyme subunit beta, 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 enz [...] (486 aa) |
| | PEX5 | Peroxisomal targeting signal 1 receptor; Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. (640 aa) |
| | FTCD | Formimidoyltransferase cyclodeaminase. (625 aa) |
| | ENSBTAP00000071610 | Acyl-CoA_dh_1 domain-containing protein. (216 aa) |
| | ENSBTAP00000072026 | Cysteine dioxygenase. (189 aa) |
| | BCAT2 | Branched-chain-amino-acid aminotransferase, mitochondrial; Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids (By similarity). (406 aa) |
| | SLC25A44 | Solute carrier family 25 member 44; Belongs to the mitochondrial carrier (TC 2.A.29) family. (361 aa) |
| | DDO | D-aspartate oxidase; Belongs to the DAMOX/DASOX family. (341 aa) |
| | NPL | N-acetylneuraminate lyase; Catalyzes the cleavage of N-acetylneuraminic acid (sialic acid) to form pyruvate and N-acetylmannosamine via a Schiff base intermediate. It prevents sialic acids from being recycled and returning to the cell surface. Involved in the N-glycolylneuraminic acid (Neu5Gc) degradation pathway (By similarity). (388 aa) |
| | ECHS1 | Enoyl-CoA hydratase, mitochondrial; Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate (By similarity). Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate (By similarity); Belongs to the enoyl-CoA hydratase/isomerase family. (341 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) |
| | FAH | Fumarylacetoacetase. (471 aa) |
| | ENSBTAP00000074447 | 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. (191 aa) |
| | SARDH | Sarcosine dehydrogenase. (954 aa) |
| | ACADSB | Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2- methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent (By similarity). (432 aa) |
| | ACAA1 | Mitochondrial acetyl-Coenzyme A acyltransferase 1; Belongs to the thiolase-like superfamily. Thiolase family. (424 aa) |
| | ABHD3 | Phospholipase ABHD3; Phospholipase that may play a role in phospholipids remodeling. May selectively cleave myristate (C14)-containing phosphatidylcholines through its predominant phospholipase 1 activity, cleaving preferentially acyl groups in sn1 position. In parallel, may have a minor phospholipase 2 activity acting on acyl groups in position sn2. In addition to (C14)-containing phosphatidylcholines, may also act on other medium-chain-containing and oxidatively truncated phospholipids. (411 aa) |
| | DAO | D-amino acid oxidase. (347 aa) |
| | ADTRP | Androgen dependent TFPI regulating protein. (230 aa) |
| | DECR1 | 2,4-dienoyl-CoA reductase 1. (329 aa) |
| | ABHD2 | Monoacylglycerol lipase ABHD2; Progesterone-dependent acylglycerol lipase that catalyzes hydrolysis of endocannabinoid arachidonoylglycerol (AG) from cell membrane. Acts as a progesterone receptor: progesterone-binding activates the acylglycerol lipase activity, mediating degradation of 1- arachidonoylglycerol (1AG) and 2-arachidonoylglycerol (2AG) to glycerol and arachidonic acid (AA). Also displays an ester hydrolase activity against acetyl ester, butanoate ester and hexadecanoate ester. Plays a key role in sperm capacitation in response to progesterone by mediating degradation of 2A [...] (425 aa) |
| | ABCD1 | ATP-binding cassette, sub-family D (ALD), member 1. (737 aa) |
| | F1MBI9_BOVIN | 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. (166 aa) |
| | CYP26A1 | Uncharacterized protein; Belongs to the cytochrome P450 family. (497 aa) |
| | BLMH | Bleomycin hydrolase; Belongs to the peptidase C1 family. (459 aa) |
| | CROT | Peroxisomal carnitine O-octanoyltransferase; Beta-oxidation of fatty acids. The highest activity concerns the C6 to C10 chain length substrate; Belongs to the carnitine/choline acetyltransferase family. (616 aa) |
| | HMGCL | Hydroxymethylglutaryl-CoA lyase, mitochondrial; Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3- methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta- hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism. (325 aa) |
| | ALDH5A1 | Succinate-semialdehyde dehydrogenase; Belongs to the aldehyde dehydrogenase family. (526 aa) |
| | MAT1A | S-adenosylmethionine synthase isoform type-1; Catalyzes the formation of S-adenosylmethionine from methionine and ATP. The reaction comprises two steps that are both catalyzed by the same enzyme: formation of S-adenosylmethionine (AdoMet) and triphosphate, and subsequent hydrolysis of the triphosphate; Belongs to the AdoMet synthase family. (396 aa) |
| | NUDT7 | Nudix hydrolase 7. (238 aa) |
| | ACADM | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; Acyl-CoA dehydrogenase specific for acyl chain lengths of 4 to 16 that catalyzes the initial step of fatty acid beta-oxidation. Utilizes the electron transfer flavoprotein (ETF) as an electron acceptor to transfer electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). (421 aa) |
| | ABCD3 | ATP binding cassette subfamily D member 3. (656 aa) |
| | DLD | Dihydrolipoyl dehydrogenase. (509 aa) |
| | GLDC | Glycine cleavage system P protein; The glycine cleavage system catalyzes the degradation of glycine. (1020 aa) |
| | TDH | L-threonine 3-dehydrogenase, mitochondrial; Catalyzes the NAD(+)-dependent oxidation of L-threonine to 2- amino-3-ketobutyrate, mediating L-threonine catabolism. (373 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) |
| | ACOX3 | Acyl-coenzyme A oxidase; Belongs to the acyl-CoA oxidase family. (700 aa) |
| | HSD17B10 | 3-hydroxyacyl-CoA dehydrogenase type-2; Mitochondrial dehydrogenase that catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha- hydroxysteroid dehydrogenase activity with androsterone. Catalyzes the third step in the beta-oxidation of fatty acids. Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids. Also exhibits 20- beta-OH and 21-OH dehydrogenase activities with C21 steroids. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD). Essential for structura [...] (261 aa) |
| | CYP2W1 | Cytochrome P450 family 2 subfamily W member 1; Belongs to the cytochrome P450 family. (493 aa) |
| | CDO1 | Cysteine dioxygenase type 1. (270 aa) |
| | SHMT1 | Serine hydroxymethyltransferase, cytosolic; Interconversion of serine and glycine. (484 aa) |
| | PEX7 | Peroxisomal biogenesis factor 7. (323 aa) |
| | AMDHD1 | Probable imidazolonepropionase. (426 aa) |
| | CPT1B | Carnitine O-palmitoyltransferase 1, muscle isoform. (771 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). (455 aa) |
| | PON1 | Paraoxonase 1. (355 aa) |
| | SLC27A4 | Solute carrier family 27 (Fatty acid transporter), member 4. (643 aa) |
| | ILVBL | Acetolactate synthase-like protein. (639 aa) |
| | HMGCLL1 | 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1. (340 aa) |
| | LEP | Leptin; Key player in the regulation of energy balance and body weight control. Once released into the circulation, has central and peripheral effects by binding LEPR, found in many tissues, which results in the activation of several major signaling pathways (By similarity). In the hypothalamus, acts as an appetite-regulating factor that induces a decrease in food intake and an increase in energy consumption by inducing anorexinogenic factors and suppressing orexigenic neuropeptides, also regulates bone mass and secretion of hypothalamo-pituitary-adrenal hormones. In the periphery, inc [...] (167 aa) |
| | NAGK | N-acetyl-D-glucosamine kinase; Converts endogenous N-acetylglucosamine (GlcNAc), a major component of complex carbohydrates, from lysosomal degradation or nutritional sources into GlcNAc 6-phosphate. Involved in the N- glycolylneuraminic acid (Neu5Gc) degradation pathway. Also has ManNAc kinase activity (By similarity). (344 aa) |
| | CPT2 | Carnitine O-palmitoyltransferase 2, mitochondrial; Belongs to the carnitine/choline acetyltransferase family. (658 aa) |
| | ABCD4 | ATP binding cassette subfamily D member 4. (606 aa) |
| | DDAH2 | N(G),N(G)-dimethylarginine dimethylaminohydrolase 2; Hydrolyzes N(G),N(G)-dimethyl-L-arginine (ADMA) and N(G)- monomethyl-L-arginine (MMA) which act as inhibitors of NOS. Has therefore a role in the regulation of nitric oxide generation (By similarity). (285 aa) |
| | PGD | 6-phosphogluconate dehydrogenase, decarboxylating; Belongs to the 6-phosphogluconate dehydrogenase family. (559 aa) |
| | ACAD8 | Isobutyryl-CoA dehydrogenase, mitochondrial; Isobutyryl-CoA dehydrogenase which catalyzes one of the steps of the valine catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA. (416 aa) |
| | CRABP1 | Cellular retinoic acid-binding protein 1; Cytosolic CRABPs may regulate the access of retinoic acid to the nuclear retinoic acid receptors. (137 aa) |
| | ACAT1 | Acetyl-CoA acetyltransferase, mitochondrial; This is one of the enzymes that catalyzes the last step of the mitochondrial beta-oxidation pathway, an aerobic process breaking down fatty acids into acetyl-CoA. Using free coenzyme A/CoA, catalyzes the thiolytic cleavage of medium- to long-chain 3-oxoacyl-CoAs into acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms. The activity of the enzyme is reversible and it can also catalyze the condensation of two acetyl-CoA molecules into acetoacetyl-CoA. Thereby, it plays a major role in ketone body metabolism. Belongs to the thiolase-l [...] (422 aa) |
| | ETFA | Electron transfer flavoprotein subunit alpha, mitochondrial; Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF- ubiquinone oxidoreductase (ETF dehydrogenase). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism. (333 aa) |
| | LOC782545 | Amino_oxidase domain-containing protein. (451 aa) |
| | ARG1 | Arginase-1. (322 aa) |
| | CYP26B1 | Cytochrome P450 26B1; Involved in the metabolism of retinoic acid (RA), rendering this classical morphogen inactive through oxidation. Involved in the specific inactivation of all-trans-retinoic acid (all-trans-RA), with a preference for the following substrates: all-trans-RA > 9-cis-RA > 13- cis-RA. Generates several hydroxylated forms of RA, including 4-OH-RA, 4-oxo-RA, and 18-OH-RA. Essential for postnatal survival. Plays a central role in germ cell development: acts by degrading RA in the developing testis, preventing STRA8 expression, thereby leading to delay of meiosis. Required [...] (512 aa) |
| | AASS | Alpha-aminoadipic semialdehyde synthase, mitochondrial; Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine- oxoglutarate reductase and saccharopine dehydrogenase activity, respectively (By similarity). (926 aa) |
| | BCKDHB | 2-oxoisovalerate dehydrogenase subunit beta, 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). (392 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) |
| | PCK2 | Phosphoenolpyruvate carboxykinase 2, mitochondrial. (640 aa) |
| | ACADL | Acyl-Coenzyme A dehydrogenase, long chain. (430 aa) |
| | ECI1 | Enoyl-CoA delta isomerase 1. (303 aa) |
| | HDC | Histidine decarboxylase; Catalyzes the biosynthesis of histamine from histidine. (658 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) |
| | GLS2 | Glutaminase 2. (602 aa) |
| | LPIN3 | Lipin 3. (850 aa) |
| | LOC507443 | Beta_elim_lyase domain-containing protein. (392 aa) |
| | SDSL | Serine dehydratase like. (329 aa) |
| | E1BDC8_BOVIN | 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. (177 aa) |
| | GAD2 | Glutamate decarboxylase 2. (585 aa) |
| | ALDH8A1 | 2-aminomuconic semialdehyde dehydrogenase; Catalyzes the NAD-dependent oxidation of 2-aminomuconic semialdehyde of the kynurenine metabolic pathway in L-tryptophan degradation. (487 aa) |
| | PIPOX | Peroxisomal sarcosine oxidase; Metabolizes sarcosine, L-pipecolic acid and L-proline. Belongs to the MSOX/MTOX family. (392 aa) |
| | GLS | Glutaminase. (603 aa) |
| | GNPDA1 | Glucosamine-6-phosphate isomerase 1; Seems to trigger calcium oscillations in mammalian eggs. These oscillations serve as the essential trigger for egg activation and early development of the embryo (By similarity); Belongs to the glucosamine/galactosamine-6-phosphate isomerase family. (289 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). Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. Alanine aminotransferase subfamily. (820 aa) |
| | PHYH | Phytanoyl-CoA dioxygenase, peroxisomal; Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. (337 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 (By similarity); Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (514 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) |
| | GAD1 | Glutamate decarboxylase 1; Catalyzes the production of GABA; Belongs to the group II decarboxylase family. (594 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) |
| | ABHD1 | Protein ABHD1. (404 aa) |
| | HSD17B4 | Hydroxysteroid (17-beta) dehydrogenase 4. (736 aa) |
| | OAT | Ornithine aminotransferase, mitochondrial. (439 aa) |
