#ID(s) interactor A ID(s) interactor B Alt. ID(s) interactor A Alt. ID(s) interactor B Alias(es) interactor A Alias(es) interactor B Interaction detection method(s) Publication 1st author(s) Publication Identifier(s) Taxid interactor A Taxid interactor B Interaction type(s) Source database(s) Interaction identifier(s) Confidence value(s) Expansion method(s) Biological role(s) interactor A Biological role(s) interactor B Experimental role(s) interactor A Experimental role(s) interactor B Type(s) interactor A Type(s) interactor B Xref(s) interactor A Xref(s) interactor B Interaction Xref(s) Annotation(s) interactor A Annotation(s) interactor B Interaction annotation(s) Host organism(s) Interaction parameter(s) Creation date Update date Checksum(s) interactor A Checksum(s) interactor B Interaction Checksum(s) Negative Feature(s) interactor A Feature(s) interactor B Stoichiometry(s) interactor A Stoichiometry(s) interactor B Identification method participant A Identification method participant B uniprotkb:P07686 uniprotkb:P06865 intact:EBI-7133736|ensembl:ENSP00000261416|intact:MINT-4528380 intact:EBI-723519|ensembl:ENSP00000268097|uniprotkb:Q53HS8|uniprotkb:Q6AI32|uniprotkb:B4DKE7|uniprotkb:E7ENH7 psi-mi:hexb_human(display_long)|uniprotkb:HEXB(gene name)|psi-mi:HEXB(display_short)|uniprotkb:HCC7(orf name)|uniprotkb:N-acetyl-beta-glucosaminidase subunit beta(gene name synonym)|uniprotkb:Beta-N-acetylhexosaminidase subunit beta(gene name synonym)|uniprotkb:Cervical cancer proto-oncogene 7 protein(gene name synonym) psi-mi:hexa_human(display_long)|uniprotkb:N-acetyl-beta-glucosaminidase subunit alpha(gene name synonym)|uniprotkb:Beta-N-acetylhexosaminidase subunit alpha(gene name synonym)|uniprotkb:HEXA(gene name)|psi-mi:HEXA(display_short) psi-mi:"MI:0114"(x-ray crystallography) Lemieux et al. (2006) pubmed:16698036|imex:IM-25817 taxid:9606(human)|taxid:9606(Homo sapiens) taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0407"(direct interaction) psi-mi:"MI:0469"(IntAct) intact:EBI-12592726|wwpdb:2gjx|imex:IM-25817-1 - - psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0326"(protein) psi-mi:"MI:0326"(protein) ensembl:ENSG00000049860(gene)|ensembl:ENST00000261416(transcript)|go:"GO:0001501"(skeletal system development)|go:"GO:0001669"(acrosomal vesicle)|go:"GO:0004563"(beta-N-acetylhexosaminidase activity)|go:"GO:0005576"(extracellular region)|go:"GO:0006689"(ganglioside catabolic process)|go:"GO:0006874"(cellular calcium ion homeostasis)|go:"GO:0007040"(lysosome organization)|go:"GO:0007338"(single fertilization)|go:"GO:0007341"(penetration of zona pellucida)|go:"GO:0007605"(sensory perception of sound)|go:"GO:0007626"(locomotory behavior)|go:"GO:0008049"(male courtship behavior)|go:"GO:0008360"(regulation of cell shape)|go:"GO:0008375"(acetylglucosaminyltransferase activity)|go:"GO:0008654"(phospholipid biosynthetic process)|go:"GO:0009313"(oligosaccharide catabolic process)|go:"GO:0016020"(membrane)|go:"GO:0019915"(lipid storage)|go:"GO:0030203"(glycosaminoglycan metabolic process)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0042552"(myelination)|go:"GO:0042582"(azurophil granule)|go:"GO:0042802"(identical protein binding)|go:"GO:0043202"(lysosomal lumen)|go:"GO:0043615"(astrocyte cell migration)|go:"GO:0044267"(cellular protein metabolic process)|go:"GO:0045944"(positive regulation of transcription by RNA polymerase II)|go:"GO:0048477"(oogenesis)|go:"GO:0050885"(neuromuscular process controlling balance)|go:"GO:0060473"(cortical granule)|go:"GO:0070062"(extracellular exosome)|go:"GO:0102148"(N-acetyl-beta-D-galactosaminidase activity)|interpro:IPR015883(Glycoside hydrolase, family 20, catalytic core)|interpro:IPR017853(Glycoside hydrolase, catalytic core)|interpro:IPR025705|interpro:IPR029018|interpro:IPR029019|rcsb pdb:1NOU|rcsb pdb:1NOW|rcsb pdb:1NP0|rcsb pdb:1O7A|rcsb pdb:1QBD|rcsb pdb:2GJX|rcsb pdb:2GK1|rcsb pdb:3LMY|rcsb pdb:5BRO|reactome:R-HSA-1660662|reactome:R-HSA-2022857|reactome:R-HSA-2024101|reactome:R-HSA-2160916|reactome:R-HSA-3656248|reactome:R-HSA-6798695|refseq:NP_000512.1|refseq:NP_001278933.1 refseq:NP_000511.2|ensembl:ENSG00000213614(gene)|ensembl:ENST00000268097(transcript)|go:"GO:0004563"(beta-N-acetylhexosaminidase activity)|go:"GO:0005829"(cytosol)|go:"GO:0005975"(carbohydrate metabolic process)|go:"GO:0006024"(glycosaminoglycan biosynthetic process)|go:"GO:0008375"(acetylglucosaminyltransferase activity)|go:"GO:0016020"(membrane)|go:"GO:0042582"(azurophil granule)|go:"GO:0043202"(lysosomal lumen)|go:"GO:0043231"(intracellular membrane-bounded organelle)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0070062"(extracellular exosome)|go:"GO:0102148"(N-acetyl-beta-D-galactosaminidase activity)|interpro:IPR015883(Glycoside hydrolase, family 20, catalytic core)|interpro:IPR017853(Glycoside hydrolase, catalytic core)|interpro:IPR025705|interpro:IPR029018|interpro:IPR029019|rcsb pdb:1QBC|rcsb pdb:2GJX|rcsb pdb:2GK1|reactome:R-HSA-1660662|go:"GO:0006689"(ganglioside catabolic process)|reactome:R-HSA-2024101|reactome:R-HSA-2160916|reactome:R-HSA-2022857|reactome:R-HSA-3656234|refseq:NP_001305754.1 - function:"Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2) [MIM:268800]; also known as Sandhoff disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula"|comment:mint|comment:homomint|function:Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues - figure legend:Table 1|3d-resolution:2.8A|3d-r-factors:Rw = 0.269 Rfree = 0.288|comment:Both subunits have many N-linked glycosyl residues and there are oligosaccharides bound at most of the known glycosylation sites.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2016/09/06 2016/11/03 rogid:1cc9u+fdr9Y0llAa3Aji4SrTh2k9606 rogid:vb+06UjAmegWcUOZUILPR2Ycuo09606 rigid:jCv8b0LdKBRl8JG53VmkZtMBoP0 false glycosylated residue:?-? glycosylated residue:?-? 4 4 psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant) uniprotkb:P06865 uniprotkb:P07686 intact:EBI-723519|ensembl:ENSP00000268097|uniprotkb:Q53HS8|uniprotkb:Q6AI32|uniprotkb:B4DKE7|uniprotkb:E7ENH7 intact:EBI-7133736|ensembl:ENSP00000261416|intact:MINT-4528380 psi-mi:hexa_human(display_long)|uniprotkb:N-acetyl-beta-glucosaminidase subunit alpha(gene name synonym)|uniprotkb:Beta-N-acetylhexosaminidase subunit alpha(gene name synonym)|uniprotkb:HEXA(gene name)|psi-mi:HEXA(display_short) psi-mi:hexb_human(display_long)|uniprotkb:HEXB(gene name)|psi-mi:HEXB(display_short)|uniprotkb:HCC7(orf name)|uniprotkb:N-acetyl-beta-glucosaminidase subunit beta(gene name synonym)|uniprotkb:Beta-N-acetylhexosaminidase subunit beta(gene name synonym)|uniprotkb:Cervical cancer proto-oncogene 7 protein(gene name synonym) psi-mi:"MI:0114"(x-ray crystallography) Lemieux et al. (2006) pubmed:16698036|imex:IM-25817 taxid:9606(human)|taxid:9606(Homo sapiens) taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0407"(direct interaction) psi-mi:"MI:0469"(IntAct) intact:EBI-12592751|pdbe:2gk1|imex:IM-25817-2 - psi-mi:"MI:1060"(spoke expansion) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0326"(protein) psi-mi:"MI:0326"(protein) refseq:NP_000511.2|ensembl:ENSG00000213614(gene)|ensembl:ENST00000268097(transcript)|go:"GO:0004563"(beta-N-acetylhexosaminidase activity)|go:"GO:0005829"(cytosol)|go:"GO:0005975"(carbohydrate metabolic process)|go:"GO:0006024"(glycosaminoglycan biosynthetic process)|go:"GO:0008375"(acetylglucosaminyltransferase activity)|go:"GO:0016020"(membrane)|go:"GO:0042582"(azurophil granule)|go:"GO:0043202"(lysosomal lumen)|go:"GO:0043231"(intracellular membrane-bounded organelle)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0070062"(extracellular exosome)|go:"GO:0102148"(N-acetyl-beta-D-galactosaminidase activity)|interpro:IPR015883(Glycoside hydrolase, family 20, catalytic core)|interpro:IPR017853(Glycoside hydrolase, catalytic core)|interpro:IPR025705|interpro:IPR029018|interpro:IPR029019|rcsb pdb:1QBC|rcsb pdb:2GJX|rcsb pdb:2GK1|reactome:R-HSA-1660662|go:"GO:0006689"(ganglioside catabolic process)|reactome:R-HSA-2024101|reactome:R-HSA-2160916|reactome:R-HSA-2022857|reactome:R-HSA-3656234|refseq:NP_001305754.1 ensembl:ENSG00000049860(gene)|ensembl:ENST00000261416(transcript)|go:"GO:0001501"(skeletal system development)|go:"GO:0001669"(acrosomal vesicle)|go:"GO:0004563"(beta-N-acetylhexosaminidase activity)|go:"GO:0005576"(extracellular region)|go:"GO:0006689"(ganglioside catabolic process)|go:"GO:0006874"(cellular calcium ion homeostasis)|go:"GO:0007040"(lysosome organization)|go:"GO:0007338"(single fertilization)|go:"GO:0007341"(penetration of zona pellucida)|go:"GO:0007605"(sensory perception of sound)|go:"GO:0007626"(locomotory behavior)|go:"GO:0008049"(male courtship behavior)|go:"GO:0008360"(regulation of cell shape)|go:"GO:0008375"(acetylglucosaminyltransferase activity)|go:"GO:0008654"(phospholipid biosynthetic process)|go:"GO:0009313"(oligosaccharide catabolic process)|go:"GO:0016020"(membrane)|go:"GO:0019915"(lipid storage)|go:"GO:0030203"(glycosaminoglycan metabolic process)|go:"GO:0035578"(azurophil granule lumen)|go:"GO:0042552"(myelination)|go:"GO:0042582"(azurophil granule)|go:"GO:0042802"(identical protein binding)|go:"GO:0043202"(lysosomal lumen)|go:"GO:0043615"(astrocyte cell migration)|go:"GO:0044267"(cellular protein metabolic process)|go:"GO:0045944"(positive regulation of transcription by RNA polymerase II)|go:"GO:0048477"(oogenesis)|go:"GO:0050885"(neuromuscular process controlling balance)|go:"GO:0060473"(cortical granule)|go:"GO:0070062"(extracellular exosome)|go:"GO:0102148"(N-acetyl-beta-D-galactosaminidase activity)|interpro:IPR015883(Glycoside hydrolase, family 20, catalytic core)|interpro:IPR017853(Glycoside hydrolase, catalytic core)|interpro:IPR025705|interpro:IPR029018|interpro:IPR029019|rcsb pdb:1NOU|rcsb pdb:1NOW|rcsb pdb:1NP0|rcsb pdb:1O7A|rcsb pdb:1QBD|rcsb pdb:2GJX|rcsb pdb:2GK1|rcsb pdb:3LMY|rcsb pdb:5BRO|reactome:R-HSA-1660662|reactome:R-HSA-2022857|reactome:R-HSA-2024101|reactome:R-HSA-2160916|reactome:R-HSA-3656248|reactome:R-HSA-6798695|refseq:NP_000512.1|refseq:NP_001278933.1 - - function:"Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2) [MIM:268800]; also known as Sandhoff disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula"|comment:mint|comment:homomint|function:Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues figure legend:Table 1i|3d-resolution:3.25A|3d-r-factors:Rw = 0.274 Rfree = 0.322|comment:Both subunits have many N-linked glycosyl residues and there are oligosaccharides bound at most of the known glycosylation sites.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2016/09/06 2017/08/09 rogid:vb+06UjAmegWcUOZUILPR2Ycuo09606 rogid:1cc9u+fdr9Y0llAa3Aji4SrTh2k9606 - false glycosylated residue:?-? glycosylated residue:?-? 4 4 psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant) intact:EBI-12592764 uniprotkb:P06865 chembl:CHEMBL257158 intact:EBI-723519|ensembl:ENSP00000268097|uniprotkb:Q53HS8|uniprotkb:Q6AI32|uniprotkb:B4DKE7|uniprotkb:E7ENH7 psi-mi:nag-thiazoline(display_short)|psi-mi:EBI-12592764(display_long) psi-mi:hexa_human(display_long)|uniprotkb:N-acetyl-beta-glucosaminidase subunit alpha(gene name synonym)|uniprotkb:Beta-N-acetylhexosaminidase subunit alpha(gene name synonym)|uniprotkb:HEXA(gene name)|psi-mi:HEXA(display_short) psi-mi:"MI:0114"(x-ray crystallography) Lemieux et al. (2006) pubmed:16698036|imex:IM-25817 taxid:-2(chemical synthesis)|taxid:-2("Chemical synthesis (Chemical synthesis)") taxid:9606(human)|taxid:9606(Homo sapiens) psi-mi:"MI:0407"(direct interaction) psi-mi:"MI:0469"(IntAct) intact:EBI-12592751|pdbe:2gk1|imex:IM-25817-2 - psi-mi:"MI:1060"(spoke expansion) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0499"(unspecified role) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0497"(neutral component) psi-mi:"MI:0328"(small molecule) psi-mi:"MI:0326"(protein) - refseq:NP_000511.2|ensembl:ENSG00000213614(gene)|ensembl:ENST00000268097(transcript)|go:"GO:0004563"(beta-N-acetylhexosaminidase activity)|go:"GO:0005829"(cytosol)|go:"GO:0005975"(carbohydrate metabolic process)|go:"GO:0006024"(glycosaminoglycan biosynthetic process)|go:"GO:0008375"(acetylglucosaminyltransferase activity)|go:"GO:0016020"(membrane)|go:"GO:0042582"(azurophil granule)|go:"GO:0043202"(lysosomal lumen)|go:"GO:0043231"(intracellular membrane-bounded organelle)|go:"GO:0046982"(protein heterodimerization activity)|go:"GO:0070062"(extracellular exosome)|go:"GO:0102148"(N-acetyl-beta-D-galactosaminidase activity)|interpro:IPR015883(Glycoside hydrolase, family 20, catalytic core)|interpro:IPR017853(Glycoside hydrolase, catalytic core)|interpro:IPR025705|interpro:IPR029018|interpro:IPR029019|rcsb pdb:1QBC|rcsb pdb:2GJX|rcsb pdb:2GK1|reactome:R-HSA-1660662|go:"GO:0006689"(ganglioside catabolic process)|reactome:R-HSA-2024101|reactome:R-HSA-2160916|reactome:R-HSA-2022857|reactome:R-HSA-3656234|refseq:NP_001305754.1 - - - figure legend:Table 1i|3d-resolution:3.25A|3d-r-factors:Rw = 0.274 Rfree = 0.322|comment:Both subunits have many N-linked glycosyl residues and there are oligosaccharides bound at most of the known glycosylation sites.|full coverage:Only protein-protein interactions|curation depth:imex curation taxid:-1(in vitro)|taxid:-1(In vitro) - 2016/09/06 2017/08/09 - rogid:vb+06UjAmegWcUOZUILPR2Ycuo09606 - false - glycosylated residue:?-? - 4 psi-mi:"MI:0396"(predetermined participant) psi-mi:"MI:0396"(predetermined participant)