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OTC OTC PYCR3 PYCR3 TMEM109 TMEM109 AGXT2 AGXT2 ARG2 ARG2 DPYS DPYS GLUD1 GLUD1 GADL1 GADL1 NAGS NAGS PRODH2 PRODH2 ASL ASL GLUL GLUL GLS2 GLS2 ALDH5A1 ALDH5A1 GLS GLS UPB1 UPB1 CNDP2 CNDP2 NOS2 NOS2 GLUD2 GLUD2 PYCR2 PYCR2 ALDH9A1 ALDH9A1 ARG1 ARG1 PRODH PRODH CNDP1 CNDP1 OAT OAT LGSN LGSN ALDH18A1 ALDH18A1 ASS1 ASS1 ALDH4A1 ALDH4A1 CARNMT1 CARNMT1 ABAT ABAT PYCR1 PYCR1 CARNS1 CARNS1 H7C0S8_HUMAN H7C0S8_HUMAN CPS1 CPS1 IAH1 IAH1 U3KQP1_HUMAN U3KQP1_HUMAN LOC102724788 LOC102724788 FADS6 FADS6
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splice isoforms or post-translational modifications are collapsed, i.e. each node represents all the proteins produced by a single, protein-coding gene locus.
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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
filled nodes:
a 3D structure is known or predicted
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Known Interactions
from curated databases
experimentally determined
Predicted Interactions
gene neighborhood
gene fusions
gene co-occurrence
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textmining
co-expression
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OTCOrnithine carbamoyltransferase, mitochondrial; Ornithine carbamoyltransferase; Belongs to the aspartate/ornithine carbamoyltransferase superfamily. OTCase family. (354 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)
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)
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)
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)
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)
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)
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)
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)
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)
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)
CNDP1Carnosine dipeptidase 1; Belongs to the peptidase M20A family. (507 aa)
OATOrnithine aminotransferase, mitochondrial; Ornithine aminotransferase. (439 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)
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)
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)
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)
IAH1Isoamyl acetate-hydrolyzing esterase 1 homolog; Probable lipase; Belongs to the 'GDSL' lipolytic enzyme family. IAH1 subfamily. (248 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)
Your Current Organism:
Homo sapiens
NCBI taxonomy Id: 9606
Other names: H. sapiens, human, man
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