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MTO1 MTO1 FOLR1 FOLR1 GLDC GLDC RFK RFK MTHFR MTHFR TXN TXN MCU MCU IBA57 IBA57 MTR MTR GTPBP3 GTPBP3 SHMT2 SHMT2 DHFR2 DHFR2 SHMT1 SHMT1 SFXN1 SFXN1 TYMS TYMS SLC19A1 SLC19A1 FOLR2 FOLR2 SLC25A32 SLC25A32 FLAD1 FLAD1 QDPR QDPR ALDH1L1 ALDH1L1 MTFMT MTFMT ATIC ATIC ALDH1L2 ALDH1L2 MTHFS MTHFS GGH GGH SLC25A38 SLC25A38 SLC46A1 SLC46A1 MTHFD1L MTHFD1L DHFR DHFR
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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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experimentally determined
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MTO1Protein MTO1 homolog, mitochondrial; Involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs. Belongs to the MnmG family. (732 aa)
FOLR1Folate receptor alpha; Binds to folate and reduced folic acid derivatives and mediates delivery of 5-methyltetrahydrofolate and folate analogs into the interior of cells. Has high affinity for folate and folic acid analogs at neutral pH. Exposure to slightly acidic pH after receptor endocytosis triggers a conformation change that strongly reduces its affinity for folates and mediates their release. Required for normal embryonic development and normal cell proliferation. (257 aa)
GLDCGlycine dehydrogenase (decarboxylating), mitochondrial; The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH). (1020 aa)
RFKRiboflavin kinase; Catalyzes the phosphorylation of riboflavin (vitamin B2) to form flavin-mononucleotide (FMN), hence rate-limiting enzyme in the synthesis of FAD. Essential for TNF-induced reactive oxygen species (ROS) production. Through its interaction with both TNFRSF1A and CYBA, physically and functionally couples TNFRSF1A to NADPH oxidase. TNF- activation of RFK may enhance the incorporation of FAD in NADPH oxidase, a critical step for the assembly and activation of NADPH oxidase. (155 aa)
MTHFRMethylenetetrahydrofolate reductase; Catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a co-substrate for homocysteine remethylation to methionine. (697 aa)
TXNThioredoxin; Participates in various redox reactions through the reversible oxidation of its active center dithiol to a disulfide and catalyzes dithiol-disulfide exchange reactions. Plays a role in the reversible S- nitrosylation of cysteine residues in target proteins, and thereby contributes to the response to intracellular nitric oxide. Nitrosylates the active site Cys of CASP3 in response to nitric oxide (NO), and thereby inhibits caspase-3 activity. Induces the FOS/JUN AP-1 DNA-binding activity in ionizing radiation (IR) cells through its oxidation/reduction status and stimulates [...] (105 aa)
MCUCalcium uniporter protein, mitochondrial; Mitochondrial inner membrane calcium uniporter that mediates calcium uptake into mitochondria. Constitutes the pore-forming and calcium-conducting subunit of the uniporter complex (uniplex). Activity is regulated by MICU1 and MICU2. At low Ca(2+) levels MCU activity is down-regulated by MICU1 and MICU2; at higher Ca(2+) levels MICU1 increases MCU activity. Mitochondrial calcium homeostasis plays key roles in cellular physiology and regulates cell bioenergetics, cytoplasmic calcium signals and activation of cell death pathways. Involved in buffe [...] (351 aa)
IBA57Putative transferase CAF17, mitochondrial; Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway. (356 aa)
MTRMethionine synthase; Catalyzes the transfer of a methyl group from methyl- cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofolate (By similarity); Belongs to the vitamin-B12 dependent methionine synthase family. (1265 aa)
GTPBP3tRNA modification GTPase GTPBP3, mitochondrial; GTPase involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs. (524 aa)
SHMT2Serine hydroxymethyltransferase, mitochondrial; Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis. 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)
DHFR2Dihydrofolate reductase 2, mitochondrial; Key enzyme in folate metabolism. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. Required to prevent uracil accumulation in mtDNA. Binds its own mRNA and that of DHFR. (187 aa)
SHMT1Serine hydroxymethyltransferase, cytosolic; Interconversion of serine and glycine. (483 aa)
SFXN1Sideroflexin-1; Mitochondrial serine transporter that mediates transport of serine into mitochondria, an important step of the one-carbon metabolism pathway. Mitochondrial serine is converted to glycine and formate, which then exits to the cytosol where it is used to generate the charged folates that serve as one-carbon donors. Transports both D-serine and L-serine. Also able to transport other amino-acids, such as alanine. May be indirectly involved in the transport of a component required for iron utilization into or out of the mitochondria (By similarity); Belongs to the sideroflexi [...] (322 aa)
TYMSThymidylate synthase; Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. (313 aa)
SLC19A1Reduced folate transporter; Transporter that mediates the import of reduced folates and a subset of cyclic dinucleotides. Has high affinity for N5-methyltetrahydrofolate, the predominant circulating form of folate. Also able to mediate the import of antifolate drug methotrexate. Acts as an importer of immunoreactive cyclic dinucleotides, such as cyclic GMP-AMP (2'-3'-cGAMP), an immune messenger produced in response to DNA virus in the cytosol, and its linkage isomer 3'-3'-cGAMP. Mechanistically, acts as an antiporter, which export of intracellular organic anions to facilitate uptake of [...] (591 aa)
FOLR2Folate receptor beta; Binds to folate and reduced folic acid derivatives and mediates delivery of 5-methyltetrahydrofolate and folate analogs into the interior of cells. Has high affinity for folate and folic acid analogs at neutral pH. Exposure to slightly acidic pH after receptor endocytosis triggers a conformation change that strongly reduces its affinity for folates and mediates their release. (255 aa)
SLC25A32Mitochondrial folate transporter/carrier; Transports folate across the inner membranes of mitochondria. Can also transport FAD across the mitochondrial inner membrane. (315 aa)
FLAD1Molybdenum cofactor biosynthesis protein-like region; Catalyzes the adenylation of flavin mononucleotide (FMN) to form flavin adenine dinucleotide (FAD) coenzyme. In the C-terminal section; belongs to the PAPS reductase family. FAD1 subfamily. (587 aa)
QDPRDihydropteridine 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. (244 aa)
ALDH1L1Cytosolic 10-formyltetrahydrofolate dehydrogenase; Aldehyde dehydrogenase 1 family member L1; In the N-terminal section; belongs to the GART family. (912 aa)
MTFMTMethionyl-tRNA formyltransferase, mitochondrial; Formylates methionyl-tRNA in mitochondria. A single tRNA(Met) gene gives rise to both an initiator and an elongator species via an unknown mechanism (By similarity). (389 aa)
ATICBifunctional purine biosynthesis protein PURH, N-terminally processed; Bifunctional enzyme that catalyzes 2 steps in purine biosynthesis; Belongs to the PurH family. (592 aa)
ALDH1L2Mitochondrial 10-formyltetrahydrofolate dehydrogenase; Aldehyde dehydrogenase 1 family member L2; In the N-terminal section; belongs to the GART family. (923 aa)
MTHFS5-formyltetrahydrofolate cyclo-ligase; Contributes to tetrahydrofolate metabolism. Helps regulate carbon flow through the folate-dependent one-carbon metabolic network that supplies carbon for the biosynthesis of purines, thymidine and amino acids. Catalyzes the irreversible conversion of 5- formyltetrahydrofolate (5-FTHF) to yield 5,10-methenyltetrahydrofolate. (203 aa)
GGHGamma-glutamyl hydrolase; Hydrolyzes the polyglutamate sidechains of pteroylpolyglutamates. Progressively removes gamma-glutamyl residues from pteroylpoly-gamma-glutamate to yield pteroyl-alpha-glutamate (folic acid) and free glutamate. May play an important role in the bioavailability of dietary pteroylpolyglutamates and in the metabolism of pteroylpolyglutamates and antifolates; Belongs to the peptidase C26 family. (318 aa)
SLC25A38Mitochondrial glycine transporter; Mitochondrial glycine transporter that imports glycine into the mitochondrial matrix. Plays an important role in providing glycine for the first enzymatic step in heme biosynthesis, the condensation of glycine with succinyl-CoA to produce 5-aminolevulinate (ALA) in the mitochondrial matrix. Required during erythropoiesis. Belongs to the mitochondrial carrier (TC 2.A.29) family. SLC25A38 subfamily. (304 aa)
SLC46A1Proton-coupled folate transporter; Has been shown to act both as an intestinal proton-coupled high-affinity folate transporter and as an intestinal heme transporter which mediates heme uptake from the gut lumen into duodenal epithelial cells. The iron is then released from heme and may be transported into the bloodstream. Dietary heme iron is an important nutritional source of iron. Shows a higher affinity for folate than heme. Belongs to the major facilitator superfamily. SLC46A family. (459 aa)
MTHFD1LMonofunctional C1-tetrahydrofolate synthase, mitochondrial; May provide the missing metabolic reaction required to link the mitochondria and the cytoplasm in the mammalian model of one-carbon folate metabolism in embryonic an transformed cells complementing thus the enzymatic activities of MTHFD2; In the N-terminal section; belongs to the tetrahydrofolate dehydrogenase/cyclohydrolase family. (979 aa)
DHFRDihydrofolate reductase; Key enzyme in folate metabolism. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. Catalyzes an essential reaction for de novo glycine and purine synthesis, and for DNA precursor synthesis. Binds its own mRNA and that of DHFR2. (187 aa)
Your Current Organism:
Homo sapiens
NCBI taxonomy Id: 9606
Other names: H. sapiens, human, man
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