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OTC OTC DHODH DHODH PYCR3 PYCR3 TMEM109 TMEM109 GOLT1B GOLT1B AGXT2 AGXT2 ACY3 ACY3 FOLH1 FOLH1 ARG2 ARG2 ASPA ASPA CAD CAD DPYS DPYS GLUD1 GLUD1 GADL1 GADL1 DPYSL5 DPYSL5 NAGS NAGS PRODH2 PRODH2 SLC23A1 SLC23A1 ASL ASL GLUL GLUL GLS2 GLS2 PHYKPL PHYKPL ALDH5A1 ALDH5A1 GLS GLS CRMP1 CRMP1 UPB1 UPB1 CNDP2 CNDP2 NOS2 NOS2 GLUD2 GLUD2 FES FES TASP1 TASP1 DPYSL4 DPYSL4 PYCR2 PYCR2 DPYSL3 DPYSL3 ALDH9A1 ALDH9A1 ARG1 ARG1 PRODH PRODH RIMKLB RIMKLB PBLD PBLD CNDP1 CNDP1 OAT OAT CYB5R4 CYB5R4 LGSN LGSN ALDH18A1 ALDH18A1 ASS1 ASS1 ALDH4A1 ALDH4A1 CARNMT1 CARNMT1 SLC23A2 SLC23A2 HYKK HYKK ASPDH ASPDH ASNS ASNS ABAT ABAT ACY1 ACY1 PYCR1 PYCR1 CARNS1 CARNS1 H7C0S8_HUMAN H7C0S8_HUMAN ASRGL1 ASRGL1 CPS1 CPS1 AASS AASS NAT8L NAT8L RIMKLA RIMKLA IAH1 IAH1 DPYSL2 DPYSL2 CYB5RL CYB5RL CYB5R2 CYB5R2 TMEM116 TMEM116 ASPG ASPG U3KQP1_HUMAN U3KQP1_HUMAN LOC102724788 LOC102724788 FADS6 FADS6 ABHD14A-ACY1 ABHD14A-ACY1
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query proteins and first shell of interactors
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second shell of interactors
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proteins of unknown 3D structure
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a 3D structure is known or predicted
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from curated databases
experimentally determined
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gene neighborhood
gene fusions
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OTCOrnithine carbamoyltransferase, mitochondrial; Ornithine carbamoyltransferase; Belongs to the aspartate/ornithine carbamoyltransferase superfamily. OTCase family. (354 aa)
DHODHDihydroorotate dehydrogenase (quinone), mitochondrial; Catalyzes the conversion of dihydroorotate to orotate with quinone as electron acceptor. (395 aa)
PYCR3Pyrroline-5-carboxylate reductase 3; Enzyme that catalyzes the last step in proline biosynthesis. Proline is synthesized from either glutamate or ornithine; both are converted to pyrroline-5-carboxylate (P5C), and then to proline via pyrroline-5-carboxylate reductases (PYCRs). PYCRL is exclusively linked to the conversion of ornithine to proline. (286 aa)
TMEM109Transmembrane protein 109; May mediate cellular response to DNA damage by protecting against ultraviolet C-induced cell death. Can form voltage-gated calcium and potassium channels in vitro (By similarity). (243 aa)
GOLT1BVesicle transport protein GOT1B; May be involved in fusion of ER-derived transport vesicles with the Golgi complex. (138 aa)
AGXT2Alanine--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; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (514 aa)
ACY3N-acyl-aromatic-L-amino acid amidohydrolase (carboxylate-forming); Plays an important role in deacetylating mercapturic acids in kidney proximal tubules. Also acts on N-acetyl-aromatic amino acids (By similarity); Belongs to the AspA/AstE family. Aspartoacylase subfamily. (319 aa)
FOLH1Glutamate carboxypeptidase 2; Has both folate hydrolase and N-acetylated-alpha-linked- acidic dipeptidase (NAALADase) activity. Has a preference for tri- alpha-glutamate peptides. In the intestine, required for the uptake of folate. In the brain, modulates excitatory neurotransmission through the hydrolysis of the neuropeptide, N-aceylaspartylglutamate (NAAG), thereby releasing glutamate. Involved in prostate tumor progression. (750 aa)
ARG2Arginase-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 CD4(+) and CD8(+) T cells. May suppress inflammation- related signaling in asthmatic airway epithelium. May contribute to the immune evasion of H.pylori by restricting [...] (354 aa)
ASPAAspartoacylase; Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids. (313 aa)
CADGlutamine-dependent carbamoyl-phosphate synthase; This protein is a 'fusion' protein encoding four enzymatic activities of the pyrimidine pathway (GATase, CPSase, ATCase and DHOase); In the central section; belongs to the metallo-dependent hydrolases superfamily. DHOase family. CAD subfamily. (2225 aa)
DPYSDihydropyrimidinase; Catalyzes the second step of the reductive pyrimidine degradation, the reversible hydrolytic ring opening of dihydropyrimidines. Can catalyze the ring opening of 5,6-dihydrouracil to N-carbamyl-alanine and of 5,6-dihydrothymine to N-carbamyl-amino isobutyrate. (519 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 (By similarity). (558 aa)
GADL1Acidic amino acid decarboxylase GADL1; May catalyze the decarboxylation of L-aspartate, 3-sulfino-L- alanine (cysteine sulfinic acid), and L-cysteate to beta-alanine, hypotaurine and taurine, respectively. Does not exhibit any decarboxylation activity toward glutamate. (521 aa)
DPYSL5Dihydropyrimidinase-related protein 5; May have a function in neuronal differentiation and/or axon growth; Belongs to the metallo-dependent hydrolases superfamily. Hydantoinase/dihydropyrimidinase family. (564 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. (534 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. (536 aa)
SLC23A1Solute carrier family 23 member 1; Sodium/ascorbate cotransporter. Mediates electrogenic uptake of vitamin C, with a stoichiometry of 2 Na(+) for each ascorbate; Belongs to the xanthine/uracil permease family. Nucleobase:cation symporter-2 (NCS2) (TC 2.A.40) subfamily. (602 aa)
ASLArgininosuccinate lyase. (464 aa)
GLULGlutamine synthetase; Glutamine synthetase that catalyzes the ATP-dependent conversion of glutamate and ammonia to glutamine. 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 (By similarity). Essential for proliferation of fetal skin fibroblasts. Independently of its glutamine synthetase activity, required for endothelial cell migration during vascular development: acts by regulating membrane locali [...] (373 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)
PHYKPL5-phosphohydroxy-L-lysine phospho-lyase; Catalyzes the pyridoxal-phosphate-dependent breakdown of 5- phosphohydroxy-L-lysine, converting it to ammonia, inorganic phosphate and 2-aminoadipate semialdehyde. (450 aa)
ALDH5A1Succinate-semialdehyde dehydrogenase, mitochondrial; Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA). (548 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 ; Belongs to the glutaminase family. (669 aa)
CRMP1Dihydropyrimidinase-related protein 1; Necessary for signaling by class 3 semaphorins and subsequent remodeling of the cytoskeleton. Plays a role in axon guidance. During the axon guidance process, acts downstream of SEMA3A to promote FLNA dissociation from F-actin which results in the rearrangement of the actin cytoskeleton and the collapse of the growth cone. Involved in invasive growth and cell migration. May participate in cytokinesis. (686 aa)
UPB1Beta-ureidopropionase; Catalyzes a late step in pyrimidine degradation. Converts N-carbamoyl-beta-alanine (3-ureidopropanoate) into beta-alanine, ammonia and carbon dioxide. Likewise, converts N-carbamoyl-beta- aminoisobutyrate (3-ureidoisobutyrate) into beta-aminoisobutyrate, ammonia and carbon dioxide (Probable). Belongs to the carbon-nitrogen hydrolase superfamily. BUP family. (384 aa)
CNDP2Cytosolic non-specific dipeptidase; Hydrolyzes a variety of dipeptides including L-carnosine but has a strong preference for Cys-Gly. Acts as a functional tumor suppressor in gastric cancer via activation of the mitogen-activated protein kinase (MAPK) pathway. An elevated level of CNDP2 activates the p38 and JNK MAPK pathways to induce cell apoptosis, and a lower level of CNDP2 activates the ERK MAPK pathway to promote cell proliferation. Isoform 2 may play a role as tumor suppressor in hepatocellular carcinoma (HCC) cells. Catalyzes the production of N-lactoyl-amino acids from lactate [...] (475 aa)
NOS2Nitric 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 (By similarity). As component of the iNOS-S100A8/9 transnitrosylase complex involved in the selective inflammatory stimulus-dependent S-nitrosylation of GAPDH on 'Cys-247' implicated in regulation of the GAIT complex activity and probably multiple targets including ANXA5, EZR [...] (1153 aa)
GLUD2Glutamate dehydrogenase 2, mitochondrial; Important for recycling the chief excitatory neurotransmitter, glutamate, during neurotransmission. (558 aa)
FESTyrosine-protein kinase Fes/Fps; Tyrosine-protein kinase that acts downstream of cell surface receptors and plays a role in the regulation of the actin cytoskeleton, microtubule assembly, cell attachment and cell spreading. Plays a role in FCER1 (high affinity immunoglobulin epsilon receptor)-mediated signaling in mast cells. Acts down-stream of the activated FCER1 receptor and the mast/stem cell growth factor receptor KIT. Plays a role in the regulation of mast cell degranulation. Plays a role in the regulation of cell differentiation and promotes neurite outgrowth in response to NGF [...] (822 aa)
TASP1Threonine aspartase subunit alpha; Protease involved in KMT2A/MLL1 processing and, consequently, in the correct expression of the early HOXA gene cluster. Belongs to the Ntn-hydrolase family. (420 aa)
DPYSL4Dihydropyrimidinase-related protein 4; Necessary for signaling by class 3 semaphorins and subsequent remodeling of the cytoskeleton. Plays a role in axon guidance, neuronal growth cone collapse and cell migration (By similarity); Belongs to the metallo-dependent hydrolases superfamily. Hydantoinase/dihydropyrimidinase family. (572 aa)
PYCR2Pyrroline-5-carboxylate reductase 2; Housekeeping enzyme that catalyzes the last step in proline biosynthesis. In some cell types, such as erythrocytes, its primary function may be the generation of NADP(+). Can utilize both NAD and NADP. Has higher affinity for NADP, but higher catalytic efficiency with NADH. Involved in cellular response to oxidative stress. Belongs to the pyrroline-5-carboxylate reductase family. (320 aa)
DPYSL3Dihydropyrimidinase-related protein 3; Necessary for signaling by class 3 semaphorins and subsequent remodeling of the cytoskeleton. Plays a role in axon guidance, neuronal growth cone collapse and cell migration (By similarity). (684 aa)
ALDH9A14-trimethylaminobutyraldehyde dehydrogenase, N-terminally processed; Converts gamma-trimethylaminobutyraldehyde into gamma- butyrobetaine with high efficiency (in vitro). Can catalyze the irreversible oxidation of a broad range of aldehydes to the corresponding acids in an NAD-dependent reaction, but with low efficiency. (518 aa)
ARG1Arginase-1; Key element of the urea cycle converting L-arginine to urea and L-ornithine, which is further metabolized into metabolites proline and polyamides that drive collagen synthesis and bioenergetic pathways critical for cell proliferation, respectively; the urea cycle takes place primarily in the liver and, to a lesser extent, in the kidneys. Belongs to the arginase family. (330 aa)
PRODHProline dehydrogenase; Converts proline to delta-1-pyrroline-5-carboxylate. (600 aa)
RIMKLBBeta-citrylglutamate synthase B; Catalyzes the synthesis of beta-citryl-L-glutamate and N- acetyl-L-aspartyl-L-glutamate. Beta-citryl-L-glutamate is synthesized more efficiently than N-acetyl-L-aspartyl-L-glutamate. Belongs to the RimK family. (386 aa)
PBLDPhenazine biosynthesis like protein domain containing. (288 aa)
CNDP1Carnosine dipeptidase 1; Belongs to the peptidase M20A family. (507 aa)
OATOrnithine aminotransferase, mitochondrial; Ornithine aminotransferase. (439 aa)
CYB5R4Cytochrome b5 reductase 4; NADH-cytochrome b5 reductase involved in endoplasmic reticulum stress response pathway. Plays a critical role in protecting pancreatic beta-cells against oxidant stress, possibly by protecting the cell from excess buildup of reactive oxygen species (ROS). Reduces a variety of substrates in vitro, such as cytochrome c, feericyanide and methemoglobin. (521 aa)
LGSNLengsin; May act as a component of the cytoskeleton or as a chaperone for the reorganization of intermediate filament proteins during terminal differentiation in the lens. Does not seem to have enzymatic activity (By similarity); Belongs to the glutamine synthetase family. (509 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. (795 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; Belongs to the argininosuccinate synthase family. Type 1 subfamily. (412 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. (563 aa)
CARNMT1Carnosine N-methyltransferase; N-methyltransferase that catalyzes the formation of anserine (beta-alanyl-N(Pi)-methyl-L-histidine) from carnosine. Anserine, a methylated derivative of carnosine (beta-alanyl-L-histidine), is an abundant constituent of vertebrate skeletal muscles. Also methylates other L-histidine-containing di- and tripeptides such as Gly-Gly-His, Gly-His and homocarnosine (GABA-His). (409 aa)
SLC23A2Solute carrier family 23 member 2; Sodium/ascorbate cotransporter. Mediates electrogenic uptake of vitamin C, with a stoichiometry of 2 Na(+) for each ascorbate. (650 aa)
HYKKHydroxylysine kinase; Catalyzes the GTP-dependent phosphorylation of 5-hydroxy-L- lysine. (373 aa)
ASPDHPutative L-aspartate dehydrogenase; Specifically catalyzes the NAD or NADP-dependent dehydrogenation of L-aspartate to iminoaspartate. (283 aa)
ASNSAsparagine synthetase. (561 aa)
ABAT4-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; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (500 aa)
ACY1Aminoacylase-1; Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate); Belongs to the peptidase M20A family. (408 aa)
PYCR1Pyrroline-5-carboxylate reductase 1, mitochondrial; Housekeeping enzyme that catalyzes the last step in proline biosynthesis. Can utilize both NAD and NADP, but has higher affinity for NAD. Involved in the cellular response to oxidative stress. Belongs to the pyrroline-5-carboxylate reductase family. (346 aa)
CARNS1Carnosine synthase 1; Catalyzes the synthesis of carnosine and homocarnosine. Carnosine is synthesized more efficiently than homocarnosine. (950 aa)
H7C0S8_HUMANRPOL4c domain-containing protein. (307 aa)
ASRGL1Isoaspartyl 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)
CPS1Carbamoyl-phosphate synthase [ammonia], mitochondrial; Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. (1506 aa)
AASSAlpha-aminoadipic semialdehyde synthase, mitochondrial; Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine- ketoglutarate reductase and saccharopine dehydrogenase activity, respectively. (926 aa)
NAT8LN-acetylaspartate synthetase; Plays a role in the regulation of lipogenesis by producing N- acetylaspartate acid (NAA), a brain-specific metabolite. NAA occurs in high concentration in brain and its hydrolysis plays a significant part in the maintenance of intact white matter. Promotes dopamine uptake by regulating TNF-alpha expression. Attenuates methamphetamine-induced inhibition of dopamine uptake. (302 aa)
RIMKLAN-acetylaspartylglutamate synthase A; Catalyzes the synthesis of N-acetyl-L-aspartyl-L-glutamate (NAAG) and N-acetyl-L-aspartyl-L-glutamyl-L-glutamate. Belongs to the RimK family. (391 aa)
IAH1Isoamyl acetate-hydrolyzing esterase 1 homolog; Probable lipase; Belongs to the 'GDSL' lipolytic enzyme family. IAH1 subfamily. (248 aa)
DPYSL2Dihydropyrimidinase-related protein 2; Plays a role in neuronal development and polarity, as well as in axon growth and guidance, neuronal growth cone collapse and cell migration. Necessary for signaling by class 3 semaphorins and subsequent remodeling of the cytoskeleton. May play a role in endocytosis; Belongs to the metallo-dependent hydrolases superfamily. Hydantoinase/dihydropyrimidinase family. (677 aa)
CYB5RLNADH-cytochrome b5 reductase-like; NADH-cytochrome b5 reductases are involved in desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism, and, in erythrocyte, methemoglobin reduction. Belongs to the flavoprotein pyridine nucleotide cytochrome reductase family. (315 aa)
CYB5R2NADH-cytochrome b5 reductase 2; NADH-cytochrome b5 reductases are involved in desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism, and, in erythrocyte, methemoglobin reduction (By similarity). Responsible for NADH-dependent lucigenin chemiluminescence in spermatozoa by reducing both lucigenin and 2-[4-iodophenyl]-3-[4- nitrophenyl]-5-[2,4-disulfophenyl]-2H tetrazolium monosodium salt (WST- 1); Belongs to the flavoprotein pyridine nucleotide cytochrome reductase family. (276 aa)
TMEM116Transmembrane protein 116. (337 aa)
ASPG1-alkyl-2-acetylglycerophosphocholine esterase; Exhibits lysophospholipase, transacylase, PAF acetylhydrolase and asparaginase activities (By similarity). Can catalyze three types of transacylation reactions: (1) acyl transfer from 1-acyl-sn-glycero- 3-phosphocholine (1-acyl-GPC) to the sn-1(3) positions of glycerol and 2-acylglycerol (sn-1 to -1(3) transfer), (2) acyl transfer from 1-acyl- GPC to the sn-2 positions of 1-acyl-GPC, 1-acyl-sn-glycero-3- phosphoethanolamine (1-acyl-GPE), and other lysophospholipids (sn-1 to -2 transfer) and (3) acyl transfer from 2-acyl-GPC to the sn-1 po [...] (573 aa)
U3KQP1_HUMANUncharacterized protein. (246 aa)
LOC102724788Proline dehydrogenase 1, mitochondrial; Converts proline to delta-1-pyrroline-5-carboxylate. Belongs to the proline oxidase family. (600 aa)
FADS6Fatty acid desaturase 6. (368 aa)
ABHD14A-ACY1ABHD14A-ACY1 readthrough. (586 aa)
Your Current Organism:
Homo sapiens
NCBI taxonomy Id: 9606
Other names: H. sapiens, human, man
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