node1 | node2 | node1 accession | node2 accession | node1 annotation | node2 annotation | score |
AASS | BTD | ENSP00000403768 | ENSP00000500193 | 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- ketoglutarate reductase and saccharopine dehydrogenase activity, respectively. | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | 0.819 |
ACACA | BTD | ENSP00000483300 | ENSP00000500193 | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | 0.622 |
ACACA | HLCS | ENSP00000483300 | ENSP00000382071 | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase. | 0.961 |
ACACA | MCCC1 | ENSP00000483300 | ENSP00000265594 | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. | 0.749 |
ACACA | MCCC2 | ENSP00000483300 | ENSP00000343657 | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism; Belongs to the AccD/PCCB family. | 0.840 |
ACACA | PC | ENSP00000483300 | ENSP00000498994 | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | Pyruvate carboxylase, mitochondrial; Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate. | 0.775 |
ACACA | PCCA | ENSP00000483300 | ENSP00000365462 | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | Propionyl-CoA carboxylase alpha chain, mitochondrial; This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domai [...] | 0.740 |
BTD | AASS | ENSP00000500193 | ENSP00000403768 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | 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- ketoglutarate reductase and saccharopine dehydrogenase activity, respectively. | 0.819 |
BTD | ACACA | ENSP00000500193 | ENSP00000483300 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | 0.622 |
BTD | HLCS | ENSP00000500193 | ENSP00000382071 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase. | 0.996 |
BTD | MCCC1 | ENSP00000500193 | ENSP00000265594 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. | 0.788 |
BTD | MCCC2 | ENSP00000500193 | ENSP00000343657 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism; Belongs to the AccD/PCCB family. | 0.814 |
BTD | PC | ENSP00000500193 | ENSP00000498994 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Pyruvate carboxylase, mitochondrial; Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate. | 0.905 |
BTD | PCCA | ENSP00000500193 | ENSP00000365462 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Propionyl-CoA carboxylase alpha chain, mitochondrial; This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domai [...] | 0.796 |
BTD | SLC19A2 | ENSP00000500193 | ENSP00000236137 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Thiamine transporter 1; High-affinity transporter for the intake of thiamine. | 0.619 |
BTD | SLC19A3 | ENSP00000500193 | ENSP00000495385 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Thiamine transporter 2; Mediates high affinity thiamine uptake, probably via a proton anti-port mechanism. Has no folate transport activity. Belongs to the reduced folate carrier (RFC) transporter (TC 2.A.48) family. | 0.732 |
BTD | USHBP1 | ENSP00000500193 | ENSP00000252597 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | Usher syndrome type-1C protein-binding protein 1; USH1 protein network component harmonin binding protein 1. | 0.812 |
HLCS | ACACA | ENSP00000382071 | ENSP00000483300 | Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase. | Acetyl-CoA carboxylase 1; Cytosolic enzyme that catalyzes the carboxylation of acetyl- CoA to malonyl-CoA, the first and rate-limiting step of de novo fatty acid biosynthesis. This is a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. | 0.961 |
HLCS | BTD | ENSP00000382071 | ENSP00000500193 | Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase. | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. | 0.996 |
HLCS | MCCC1 | ENSP00000382071 | ENSP00000265594 | Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase; Biotin--protein ligase catalyzing the biotinylation of the 4 biotin-dependent carboxylases acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, and methylcrotonyl-CoA carboxylase. | Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. | 0.984 |