node1 | node2 | node1 accession | node2 accession | node1 annotation | node2 annotation | score |
AASS | BTD | ENSP00000377040 | ENSP00000400995 | 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 | 0.820 |
ACY1 | ASPA | ENSP00000384296 | ENSP00000263080 | Aminoacylase-1; Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate); Belongs to the peptidase M20A family | Aspartoacylase; 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 | 0.908 |
ACY1 | BTD | ENSP00000384296 | ENSP00000400995 | Aminoacylase-1; Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate); Belongs to the peptidase M20A family | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | 0.674 |
ASPA | ACY1 | ENSP00000263080 | ENSP00000384296 | Aspartoacylase; 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 | Aminoacylase-1; Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate); Belongs to the peptidase M20A family | 0.908 |
ASPA | BTD | ENSP00000263080 | ENSP00000400995 | Aspartoacylase; 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 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | 0.757 |
BTD | AASS | ENSP00000400995 | ENSP00000377040 | 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 | 0.820 |
BTD | ACY1 | ENSP00000400995 | ENSP00000384296 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | Aminoacylase-1; Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate); Belongs to the peptidase M20A family | 0.674 |
BTD | ASPA | ENSP00000400995 | ENSP00000263080 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | Aspartoacylase; 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 | 0.757 |
BTD | HLCS | ENSP00000400995 | ENSP00000382071 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | Biotin--protein ligase; Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase | 0.988 |
BTD | MCCC1 | ENSP00000400995 | 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 | 0.820 |
BTD | MCCC2 | ENSP00000400995 | 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 | 0.754 |
BTD | PC | ENSP00000400995 | ENSP00000377532 | 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 | 0.816 |
BTD | PCCA | ENSP00000400995 | ENSP00000365462 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | propionyl-CoA carboxylase alpha subunit | 0.778 |
BTD | TPP1 | ENSP00000400995 | ENSP00000299427 | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | Tripeptidyl-peptidase 1; Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus (By similarity) | 0.659 |
BTD | USHBP1 | ENSP00000400995 | 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; Belongs to the MCC family | 0.757 |
HLCS | BTD | ENSP00000382071 | ENSP00000400995 | Biotin--protein ligase; Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase | Biotinidase; Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation | 0.988 |
HLCS | MCCC1 | ENSP00000382071 | ENSP00000265594 | Biotin--protein ligase; Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-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 | 0.990 |
HLCS | MCCC2 | ENSP00000382071 | ENSP00000343657 | Biotin--protein ligase; Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase | 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 | 0.947 |
HLCS | PC | ENSP00000382071 | ENSP00000377532 | Biotin--protein ligase; Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase | 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 | 0.990 |
HLCS | PCCA | ENSP00000382071 | ENSP00000365462 | Biotin--protein ligase; Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl- CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase | propionyl-CoA carboxylase alpha subunit | 0.989 |