| node1 | node2 | node1 accession | node2 accession | node1 annotation | node2 annotation | score |
| ASPA | SGSH | ENSP00000263080 | ENSP00000314606 | 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 | N-sulphoglucosamine sulphohydrolase; Catalyzes a step in lysosomal heparan sulfate degradation; Belongs to the sulfatase family | 0.667 |
| ASPA | SUMF1 | ENSP00000263080 | ENSP00000272902 | 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 | Sulfatase-modifying factor 1; Using molecular oxygen and an unidentified reducing agent, oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Known substrates include GALNS, ARSA, STS and ARSE; Belongs to the sulfatase-modifying factor family | 0.606 |
| CLCN6 | CLN5 | ENSP00000234488 | ENSP00000366673 | Chloride transport protein 6; Chloride transport protein, initially identified as voltage-gated chloride channel. The presence of the conserved gating glutamate residues suggests that is functions as antiporter; Belongs to the chloride channel (TC 2.A.49) family. ClC-6/CLCN6 subfamily | Ceroid-lipofuscinosis neuronal protein 5; Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane | 0.781 |
| CLCN6 | CLN6 | ENSP00000234488 | ENSP00000249806 | Chloride transport protein 6; Chloride transport protein, initially identified as voltage-gated chloride channel. The presence of the conserved gating glutamate residues suggests that is functions as antiporter; Belongs to the chloride channel (TC 2.A.49) family. ClC-6/CLCN6 subfamily | CLN6, transmembrane ER protein | 0.794 |
| CLCN6 | PPT1 | ENSP00000234488 | ENSP00000394863 | Chloride transport protein 6; Chloride transport protein, initially identified as voltage-gated chloride channel. The presence of the conserved gating glutamate residues suggests that is functions as antiporter; Belongs to the chloride channel (TC 2.A.49) family. ClC-6/CLCN6 subfamily | Palmitoyl-protein thioesterase 1; Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons; Belongs to the palmitoyl-protein thioesterase family | 0.565 |
| CLCN6 | SGSH | ENSP00000234488 | ENSP00000314606 | Chloride transport protein 6; Chloride transport protein, initially identified as voltage-gated chloride channel. The presence of the conserved gating glutamate residues suggests that is functions as antiporter; Belongs to the chloride channel (TC 2.A.49) family. ClC-6/CLCN6 subfamily | N-sulphoglucosamine sulphohydrolase; Catalyzes a step in lysosomal heparan sulfate degradation; Belongs to the sulfatase family | 0.777 |
| CLN5 | CLCN6 | ENSP00000366673 | ENSP00000234488 | Ceroid-lipofuscinosis neuronal protein 5; Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane | Chloride transport protein 6; Chloride transport protein, initially identified as voltage-gated chloride channel. The presence of the conserved gating glutamate residues suggests that is functions as antiporter; Belongs to the chloride channel (TC 2.A.49) family. ClC-6/CLCN6 subfamily | 0.781 |
| CLN5 | CLN6 | ENSP00000366673 | ENSP00000249806 | Ceroid-lipofuscinosis neuronal protein 5; Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane | CLN6, transmembrane ER protein | 0.934 |
| CLN5 | PPT1 | ENSP00000366673 | ENSP00000394863 | Ceroid-lipofuscinosis neuronal protein 5; Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane | Palmitoyl-protein thioesterase 1; Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons; Belongs to the palmitoyl-protein thioesterase family | 0.835 |
| CLN5 | SGSH | ENSP00000366673 | ENSP00000314606 | Ceroid-lipofuscinosis neuronal protein 5; Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane | N-sulphoglucosamine sulphohydrolase; Catalyzes a step in lysosomal heparan sulfate degradation; Belongs to the sulfatase family | 0.807 |
| CLN6 | CLCN6 | ENSP00000249806 | ENSP00000234488 | CLN6, transmembrane ER protein | Chloride transport protein 6; Chloride transport protein, initially identified as voltage-gated chloride channel. The presence of the conserved gating glutamate residues suggests that is functions as antiporter; Belongs to the chloride channel (TC 2.A.49) family. ClC-6/CLCN6 subfamily | 0.794 |
| CLN6 | CLN5 | ENSP00000249806 | ENSP00000366673 | CLN6, transmembrane ER protein | Ceroid-lipofuscinosis neuronal protein 5; Plays a role in influencing the retrograde trafficking of lysosomal sorting receptors SORT1 and IGF2R from the endosomes to the trans-Golgi network by controlling the recruitment of retromer complex to the endosomal membrane. Regulates the localization and activation of RAB7A which is required to recruit the retromer complex to the endosomal membrane | 0.934 |
| CLN6 | PPT1 | ENSP00000249806 | ENSP00000394863 | CLN6, transmembrane ER protein | Palmitoyl-protein thioesterase 1; Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons; Belongs to the palmitoyl-protein thioesterase family | 0.832 |
| CLN6 | SGSH | ENSP00000249806 | ENSP00000314606 | CLN6, transmembrane ER protein | N-sulphoglucosamine sulphohydrolase; Catalyzes a step in lysosomal heparan sulfate degradation; Belongs to the sulfatase family | 0.799 |
| HGSNAT | IDUA | ENSP00000368965 | ENSP00000247933 | Heparan-alpha-glucosaminide N-acetyltransferase; Lysosomal acetyltransferase that acetylates the non- reducing terminal alpha-glucosamine residue of intralysosomal heparin or heparan sulfate, converting it into a substrate for luminal alpha-N-acetyl glucosaminidase | Alpha-L-iduronidase; Iduronidase, alpha-L- | 0.575 |
| HGSNAT | NAGLU | ENSP00000368965 | ENSP00000225927 | Heparan-alpha-glucosaminide N-acetyltransferase; Lysosomal acetyltransferase that acetylates the non- reducing terminal alpha-glucosamine residue of intralysosomal heparin or heparan sulfate, converting it into a substrate for luminal alpha-N-acetyl glucosaminidase | Alpha-N-acetylglucosaminidase; Involved in the degradation of heparan sulfate | 0.990 |
| HGSNAT | SGSH | ENSP00000368965 | ENSP00000314606 | Heparan-alpha-glucosaminide N-acetyltransferase; Lysosomal acetyltransferase that acetylates the non- reducing terminal alpha-glucosamine residue of intralysosomal heparin or heparan sulfate, converting it into a substrate for luminal alpha-N-acetyl glucosaminidase | N-sulphoglucosamine sulphohydrolase; Catalyzes a step in lysosomal heparan sulfate degradation; Belongs to the sulfatase family | 0.969 |
| HGSNAT | SUMF1 | ENSP00000368965 | ENSP00000272902 | Heparan-alpha-glucosaminide N-acetyltransferase; Lysosomal acetyltransferase that acetylates the non- reducing terminal alpha-glucosamine residue of intralysosomal heparin or heparan sulfate, converting it into a substrate for luminal alpha-N-acetyl glucosaminidase | Sulfatase-modifying factor 1; Using molecular oxygen and an unidentified reducing agent, oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Known substrates include GALNS, ARSA, STS and ARSE; Belongs to the sulfatase-modifying factor family | 0.420 |
| IDUA | HGSNAT | ENSP00000247933 | ENSP00000368965 | Alpha-L-iduronidase; Iduronidase, alpha-L- | Heparan-alpha-glucosaminide N-acetyltransferase; Lysosomal acetyltransferase that acetylates the non- reducing terminal alpha-glucosamine residue of intralysosomal heparin or heparan sulfate, converting it into a substrate for luminal alpha-N-acetyl glucosaminidase | 0.575 |
| IDUA | MANBA | ENSP00000247933 | ENSP00000226578 | Alpha-L-iduronidase; Iduronidase, alpha-L- | Beta-mannosidase; Exoglycosidase that cleaves the single beta-linked mannose residue from the non-reducing end of all N-linked glycoprotein oligosaccharides; Mannosidases type beta | 0.494 |
