node1 | node2 | node1 accession | node2 accession | node1 annotation | node2 annotation | score |
ACSF3 | MCEE | ENSP00000479130 | ENSP00000244217 | Acyl-CoA synthetase family member 3, 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; Acyl-CoA synthetase family | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | 0.946 |
ACSF3 | MMAA | ENSP00000479130 | ENSP00000281317 | Acyl-CoA synthetase family member 3, 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; Acyl-CoA synthetase family | Methylmalonic aciduria type A protein, mitochondrial; GTPase, binds and hydrolyzes GTP. Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis. Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MUT) and reactivation of the enzyme during catalysis | 0.545 |
ACSF3 | MMADHC | ENSP00000479130 | ENSP00000389060 | Acyl-CoA synthetase family member 3, 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; Acyl-CoA synthetase family | Methylmalonic aciduria and homocystinuria type D protein, mitochondrial; Involved in cobalamin metabolism. Plays a role in regulating the biosynthesis of two coenzymes, methylcobalamin and adenosylcobalamin. Plays a role in regulating the proportion of methylcobalamin and adenosylcobalamin. Promotes oxidation of cob(II)alamin bound to MMACHC | 0.576 |
ACSF3 | MUT | ENSP00000479130 | ENSP00000274813 | Acyl-CoA synthetase family member 3, 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; Acyl-CoA synthetase family | 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. MCM has different functions in other species; Belongs to the methylmalonyl-CoA mutase family | 0.932 |
ECHDC1 | MCEE | ENSP00000436585 | ENSP00000244217 | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, 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 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | 0.960 |
ECHDC1 | MMACHC | ENSP00000436585 | ENSP00000383840 | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, 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 | Methylmalonic aciduria and homocystinuria type C protein; Catalyzes the reductive dealkylation of cyanocobalamin to cob(II)alamin, using FAD or FMN as cofactor and NADPH as cosubstrate. Can also catalyze the glutathione-dependent reductive demethylation of methylcobalamin, and, with much lower efficiency, the glutathione-dependent reductive demethylation of adenosylcobalamin. Under anaerobic conditions cob(I)alamin is the first product; it is highly reactive and is converted to aquocob(II)alamin in the presence of oxygen. Binds cyanocobalamin, adenosylcobalamin, methylcobalamin and oth [...] | 0.668 |
ECHDC1 | MUT | ENSP00000436585 | ENSP00000274813 | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, 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 | 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. MCM has different functions in other species; Belongs to the methylmalonyl-CoA mutase family | 0.567 |
ECHDC1 | PCCA | ENSP00000436585 | ENSP00000365462 | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, 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 | propionyl-CoA carboxylase alpha subunit | 0.849 |
ECHDC1 | PCCB | ENSP00000436585 | ENSP00000419027 | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, 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 | propionyl-CoA carboxylase beta subunit | 0.907 |
GCH1 | MCEE | ENSP00000419045 | ENSP00000244217 | GTP cyclohydrolase 1; Positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVECs). May be involved in dopamine synthesis. May modify pain sensitivity and persistence. Isoform GCH-1 is the functional enzyme, the potential function of the enzymatically inactive isoforms remains unknown; Belongs to the GTP cyclohydrolase I family | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | 0.757 |
GCH1 | MUT | ENSP00000419045 | ENSP00000274813 | GTP cyclohydrolase 1; Positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVECs). May be involved in dopamine synthesis. May modify pain sensitivity and persistence. Isoform GCH-1 is the functional enzyme, the potential function of the enzymatically inactive isoforms remains unknown; Belongs to the GTP cyclohydrolase I family | 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. MCM has different functions in other species; Belongs to the methylmalonyl-CoA mutase family | 0.468 |
GCH1 | SPR | ENSP00000419045 | ENSP00000234454 | GTP cyclohydrolase 1; Positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVECs). May be involved in dopamine synthesis. May modify pain sensitivity and persistence. Isoform GCH-1 is the functional enzyme, the potential function of the enzymatically inactive isoforms remains unknown; Belongs to the GTP cyclohydrolase I family | Sepiapterin reductase; Catalyzes the final one or two reductions in tetra- hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin; Belongs to the sepiapterin reductase family | 0.953 |
MCEE | ACSF3 | ENSP00000244217 | ENSP00000479130 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | Acyl-CoA synthetase family member 3, 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; Acyl-CoA synthetase family | 0.946 |
MCEE | ECHDC1 | ENSP00000244217 | ENSP00000436585 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | Ethylmalonyl-CoA decarboxylase; Decarboxylases ethylmalonyl-CoA decarboxylase, 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 | 0.960 |
MCEE | GCH1 | ENSP00000244217 | ENSP00000419045 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | GTP cyclohydrolase 1; Positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVECs). May be involved in dopamine synthesis. May modify pain sensitivity and persistence. Isoform GCH-1 is the functional enzyme, the potential function of the enzymatically inactive isoforms remains unknown; Belongs to the GTP cyclohydrolase I family | 0.757 |
MCEE | MMAA | ENSP00000244217 | ENSP00000281317 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | Methylmalonic aciduria type A protein, mitochondrial; GTPase, binds and hydrolyzes GTP. Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis. Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MUT) and reactivation of the enzyme during catalysis | 0.880 |
MCEE | MMACHC | ENSP00000244217 | ENSP00000383840 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | Methylmalonic aciduria and homocystinuria type C protein; Catalyzes the reductive dealkylation of cyanocobalamin to cob(II)alamin, using FAD or FMN as cofactor and NADPH as cosubstrate. Can also catalyze the glutathione-dependent reductive demethylation of methylcobalamin, and, with much lower efficiency, the glutathione-dependent reductive demethylation of adenosylcobalamin. Under anaerobic conditions cob(I)alamin is the first product; it is highly reactive and is converted to aquocob(II)alamin in the presence of oxygen. Binds cyanocobalamin, adenosylcobalamin, methylcobalamin and oth [...] | 0.738 |
MCEE | MMADHC | ENSP00000244217 | ENSP00000389060 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | Methylmalonic aciduria and homocystinuria type D protein, mitochondrial; Involved in cobalamin metabolism. Plays a role in regulating the biosynthesis of two coenzymes, methylcobalamin and adenosylcobalamin. Plays a role in regulating the proportion of methylcobalamin and adenosylcobalamin. Promotes oxidation of cob(II)alamin bound to MMACHC | 0.794 |
MCEE | MUT | ENSP00000244217 | ENSP00000274813 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | 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. MCM has different functions in other species; Belongs to the methylmalonyl-CoA mutase family | 0.992 |
MCEE | PCCA | ENSP00000244217 | ENSP00000365462 | Methylmalonyl-CoA epimerase, mitochondrial; methylmalonyl-CoA epimerase; Belongs to the glyoxalase I family | propionyl-CoA carboxylase alpha subunit | 0.977 |