#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
intact:EBI-23146846	uniprotkb:O75503	-	intact:EBI-1043514|ensembl:ENSP00000366673|ensembl:ENSP00000490181|uniprotkb:B3KQK7	psi-mi:cln2_chlae(display_short)|psi-mi:EBI-23146846(display_long)	psi-mi:cln5_human(display_long)|uniprotkb:CLN5(gene name)|psi-mi:CLN5(display_short)	psi-mi:"MI:0006"(anti bait coimmunoprecipitation)	Vesa et al. (2002)	pubmed:12134079|imex:IM-27573	taxid:9534(chlae)|taxid:9534(Chlorocebus aethiops)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-23212258|imex:IM-27573-1	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	uniprotkb:O14773(see-also)	ensembl:ENSG00000102805(gene)|ensembl:ENST00000377453(transcript)|ensembl:ENST00000636183(transcript)|go:"GO:0005537"(mannose binding)|go:"GO:0005764"(lysosome)|go:"GO:0005765"(lysosomal membrane)|go:"GO:0005783"(endoplasmic reticulum)|go:"GO:0005794"(Golgi apparatus)|go:"GO:0005829"(cytosol)|go:"GO:0006465"(signal peptide processing)|go:"GO:0007040"(lysosome organization)|go:"GO:0007042"(lysosomal lumen acidification)|go:"GO:0007420"(brain development)|go:"GO:0016021"(integral component of membrane)|go:"GO:0016798"(hydrolase activity, acting on glycosyl bonds)|go:"GO:0030163"(protein catabolic process)|go:"GO:0042147"(retrograde transport, endosome to Golgi)|go:"GO:0042551"(neuron maturation)|go:"GO:0048471"(perinuclear region of cytoplasm)|go:"GO:0070062"(extracellular exosome)|go:"GO:0070085"(glycosylation)|go:"GO:1904426"(positive regulation of GTP binding)|interpro:IPR026138|go:"GO:0022008"(neurogenesis)|refseq:NP_006484.1	-	-	-	figure legend:Fig. 5a|comment:"p.Tyr392* (rs386833969) and p.Glu204* are other clinical variants resulting in disruption of interaction - are not annotated here as p.Trp26* serves as the longest required to bind region."|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:9534(chlae-cos_1)|taxid:9534(Chlorocebus aethiops COS-1 cells)	-	2019/12/13	2019/12/13	-	rogid:YBJqxznEkJjC7Rdm95cpHHBPuE49606	-	false	-	mutation disrupting interaction strength:230-230(2566)|necessary binding region:343-358(553399)|mutation disrupting interaction strength:175-358	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:Q13286	uniprotkb:O75503	intact:EBI-3248760|uniprotkb:O95089|uniprotkb:B2R7J1|ensembl:ENSP00000490105|ensembl:ENSP00000353073|ensembl:ENSP00000454229|uniprotkb:Q9UP11|uniprotkb:Q9UP12|uniprotkb:O00668|uniprotkb:Q9UP09|uniprotkb:Q9UP13|uniprotkb:Q9UP14|uniprotkb:B4DXL3|uniprotkb:Q549S9	intact:EBI-1043514|ensembl:ENSP00000366673|ensembl:ENSP00000490181|uniprotkb:B3KQK7	psi-mi:cln3_human(display_long)|uniprotkb:CLN3(gene name)|psi-mi:CLN3(display_short)|uniprotkb:BTS(gene name synonym)|uniprotkb:Batten disease protein(gene name synonym)|uniprotkb:Protein CLN3(gene name synonym)	psi-mi:cln5_human(display_long)|uniprotkb:CLN5(gene name)|psi-mi:CLN5(display_short)	psi-mi:"MI:0006"(anti bait coimmunoprecipitation)	Vesa et al. (2002)	pubmed:12134079|imex:IM-27573	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-25295128|imex:IM-27573-3	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	ensembl:ENST00000359984(transcript)|ensembl:ENST00000569430(transcript)|ensembl:ENST00000636147(transcript)|go:"GO:0000139"(Golgi membrane)|go:"GO:0001508"(action potential)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0005634"(nucleus)|go:"GO:0005737"(cytoplasm)|go:"GO:0005764"(lysosome)|go:"GO:0005765"(lysosomal membrane)|go:"GO:0005769"(early endosome)|go:"GO:0005770"(late endosome)|go:"GO:0005773"(vacuole)|go:"GO:0005776"(autophagosome)|go:"GO:0005783"(endoplasmic reticulum)|go:"GO:0005794"(Golgi apparatus)|go:"GO:0005795"(Golgi stack)|ensembl:ENSG00000188603(gene)|go:"GO:0005802"(trans-Golgi network)|go:"GO:0005829"(cytosol)|go:"GO:0005886"(plasma membrane)|go:"GO:0005901"(caveola)|go:"GO:0006898"(receptor-mediated endocytosis)|go:"GO:0007040"(lysosome organization)|go:"GO:0007042"(lysosomal lumen acidification)|go:"GO:0007611"(learning or memory)|go:"GO:0008021"(synaptic vesicle)|go:"GO:0008306"(associative learning)|go:"GO:0009992"(cellular water homeostasis)|go:"GO:0010762"(regulation of fibroblast migration)|go:"GO:0015809"|go:"GO:0016021"(integral component of membrane)|go:"GO:0016243"(regulation of autophagosome size)|go:"GO:0016477"(cell migration)|go:"GO:0016485"(protein processing)|go:"GO:0048549"(positive regulation of pinocytosis)|go:"GO:0050885"(neuromuscular process controlling balance)|go:"GO:0030036"(actin cytoskeleton organization)|go:"GO:0030176"(integral component of endoplasmic reticulum membrane)|go:"GO:0031901"(early endosome membrane)|go:"GO:0031902"(late endosome membrane)|go:"GO:0032228"(regulation of synaptic transmission, GABAergic)|go:"GO:0035235"(ionotropic glutamate receptor signaling pathway)|go:"GO:0051453"(regulation of intracellular pH)|go:"GO:0035752"(lysosomal lumen pH elevation)|go:"GO:0036359"(renal potassium excretion)|go:"GO:0042133"(neurotransmitter metabolic process)|go:"GO:0042987"(amyloid precursor protein catabolic process)|go:"GO:0051480"(regulation of cytosolic calcium ion concentration)|go:"GO:0042998"(positive regulation of Golgi to plasma membrane protein transport)|go:"GO:0051489"(regulation of filopodium assembly)|go:"GO:0043005"(neuron projection)|go:"GO:0051493"(regulation of cytoskeleton organization)|go:"GO:0051861"(glycolipid binding)|go:"GO:0043066"(negative regulation of apoptotic process)|go:"GO:0051966"(regulation of synaptic transmission, glutamatergic)|go:"GO:0043086"(negative regulation of catalytic activity)|go:"GO:0043524"(negative regulation of neuron apoptotic process)|go:"GO:0055037"(recycling endosome)|go:"GO:0061024"(membrane organization)|go:"GO:0061909"(autophagosome-lysosome fusion)|go:"GO:0044754"(autolysosome)|go:"GO:0070613"(regulation of protein processing)|go:"GO:0072659"(protein localization to plasma membrane)|go:"GO:0044857"(plasma membrane raft organization)|go:"GO:0045121"(membrane raft)|go:"GO:0090160"(Golgi to lysosome transport)|go:"GO:0045861"(negative regulation of proteolysis)|go:"GO:0046474"(glycerophospholipid biosynthetic process)|go:"GO:0046836"(glycolipid transport)|go:"GO:0047496"(vesicle transport along microtubule)|go:"GO:0090384"(phagosome-lysosome docking)|go:"GO:0090385"(phagosome-lysosome fusion)|go:"GO:0048172"(regulation of short-term neuronal synaptic plasticity)|go:"GO:0048306"(calcium-dependent protein binding)|go:"GO:0097352"(autophagosome maturation)|go:"GO:0120146"(sulfatide binding)|go:"GO:1900079"(regulation of arginine biosynthetic process)|go:"GO:1901096"(regulation of autophagosome maturation)|go:"GO:1903076"(regulation of protein localization to plasma membrane)|go:"GO:1905146"(lysosomal protein catabolic process)|go:"GO:1905162"(regulation of phagosome maturation)|go:"GO:1905244"(regulation of modification of synaptic structure)|go:"GO:2001288"(positive regulation of caveolin-mediated endocytosis)|interpro:IPR003492(Batten's disease protein Cln3)|interpro:IPR018460|interpro:IPR036259|mint:Q13286|go:"GO:0106049"(regulation of cellular response to osmotic stress)|refseq:NP_001273033.1|refseq:NP_001273034.1|refseq:NP_000077.1|refseq:NP_001035897.1	ensembl:ENSG00000102805(gene)|ensembl:ENST00000377453(transcript)|ensembl:ENST00000636183(transcript)|go:"GO:0005537"(mannose binding)|go:"GO:0005764"(lysosome)|go:"GO:0005765"(lysosomal membrane)|go:"GO:0005783"(endoplasmic reticulum)|go:"GO:0005794"(Golgi apparatus)|go:"GO:0005829"(cytosol)|go:"GO:0006465"(signal peptide processing)|go:"GO:0007040"(lysosome organization)|go:"GO:0007042"(lysosomal lumen acidification)|go:"GO:0007420"(brain development)|go:"GO:0016021"(integral component of membrane)|go:"GO:0016798"(hydrolase activity, acting on glycosyl bonds)|go:"GO:0030163"(protein catabolic process)|go:"GO:0042147"(retrograde transport, endosome to Golgi)|go:"GO:0042551"(neuron maturation)|go:"GO:0048471"(perinuclear region of cytoplasm)|go:"GO:0070062"(extracellular exosome)|go:"GO:0070085"(glycosylation)|go:"GO:1904426"(positive regulation of GTP binding)|interpro:IPR026138|go:"GO:0022008"(neurogenesis)|refseq:NP_006484.1	-	-	-	figure legend:Fig. 5b|comment:"p.Tyr392* (rs386833969) and p.Glu204* are other clinical variants not showing any change in the strength of interaction - are not annotated here as p.Trp26* serves as the shortest sufficient to bind region."|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:9534(chlae-cos_1)|taxid:9534(Chlorocebus aethiops COS-1 cells)	-	2019/12/13	2019/12/16	rogid:zf0wXU6yZF+HuwwuMueb9vKIMy89606	rogid:YBJqxznEkJjC7Rdm95cpHHBPuE49606	rigid:D7pYRB0e7TTsbre3YuwtkbRs91I	false	-	mutation with no effect:230-230(2566)|sufficient binding region:1-343(553399)|mutation with no effect:175-358	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:Q13286	uniprotkb:O75503	intact:EBI-3248760|uniprotkb:O95089|uniprotkb:B2R7J1|ensembl:ENSP00000490105|ensembl:ENSP00000353073|ensembl:ENSP00000454229|uniprotkb:Q9UP11|uniprotkb:Q9UP12|uniprotkb:O00668|uniprotkb:Q9UP09|uniprotkb:Q9UP13|uniprotkb:Q9UP14|uniprotkb:B4DXL3|uniprotkb:Q549S9	intact:EBI-1043514|ensembl:ENSP00000366673|ensembl:ENSP00000490181|uniprotkb:B3KQK7	psi-mi:cln3_human(display_long)|uniprotkb:CLN3(gene name)|psi-mi:CLN3(display_short)|uniprotkb:BTS(gene name synonym)|uniprotkb:Batten disease protein(gene name synonym)|uniprotkb:Protein CLN3(gene name synonym)	psi-mi:cln5_human(display_long)|uniprotkb:CLN5(gene name)|psi-mi:CLN5(display_short)	psi-mi:"MI:0096"(pull down)	Vesa et al. (2002)	pubmed:12134079|imex:IM-27573	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0915"(physical association)	psi-mi:"MI:0469"(IntAct)	intact:EBI-25295159|imex:IM-27573-5	-	-	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0499"(unspecified role)	psi-mi:"MI:0498"(prey)	psi-mi:"MI:0496"(bait)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	ensembl:ENST00000359984(transcript)|ensembl:ENST00000569430(transcript)|ensembl:ENST00000636147(transcript)|go:"GO:0000139"(Golgi membrane)|go:"GO:0001508"(action potential)|go:"GO:0001934"(positive regulation of protein phosphorylation)|go:"GO:0005634"(nucleus)|go:"GO:0005737"(cytoplasm)|go:"GO:0005764"(lysosome)|go:"GO:0005765"(lysosomal membrane)|go:"GO:0005769"(early endosome)|go:"GO:0005770"(late endosome)|go:"GO:0005773"(vacuole)|go:"GO:0005776"(autophagosome)|go:"GO:0005783"(endoplasmic reticulum)|go:"GO:0005794"(Golgi apparatus)|go:"GO:0005795"(Golgi stack)|ensembl:ENSG00000188603(gene)|go:"GO:0005802"(trans-Golgi network)|go:"GO:0005829"(cytosol)|go:"GO:0005886"(plasma membrane)|go:"GO:0005901"(caveola)|go:"GO:0006898"(receptor-mediated endocytosis)|go:"GO:0007040"(lysosome organization)|go:"GO:0007042"(lysosomal lumen acidification)|go:"GO:0007611"(learning or memory)|go:"GO:0008021"(synaptic vesicle)|go:"GO:0008306"(associative learning)|go:"GO:0009992"(cellular water homeostasis)|go:"GO:0010762"(regulation of fibroblast migration)|go:"GO:0015809"|go:"GO:0016021"(integral component of membrane)|go:"GO:0016243"(regulation of autophagosome size)|go:"GO:0016477"(cell migration)|go:"GO:0016485"(protein processing)|go:"GO:0048549"(positive regulation of pinocytosis)|go:"GO:0050885"(neuromuscular process controlling balance)|go:"GO:0030036"(actin cytoskeleton organization)|go:"GO:0030176"(integral component of endoplasmic reticulum membrane)|go:"GO:0031901"(early endosome membrane)|go:"GO:0031902"(late endosome membrane)|go:"GO:0032228"(regulation of synaptic transmission, GABAergic)|go:"GO:0035235"(ionotropic glutamate receptor signaling pathway)|go:"GO:0051453"(regulation of intracellular pH)|go:"GO:0035752"(lysosomal lumen pH elevation)|go:"GO:0036359"(renal potassium excretion)|go:"GO:0042133"(neurotransmitter metabolic process)|go:"GO:0042987"(amyloid precursor protein catabolic process)|go:"GO:0051480"(regulation of cytosolic calcium ion concentration)|go:"GO:0042998"(positive regulation of Golgi to plasma membrane protein transport)|go:"GO:0051489"(regulation of filopodium assembly)|go:"GO:0043005"(neuron projection)|go:"GO:0051493"(regulation of cytoskeleton organization)|go:"GO:0051861"(glycolipid binding)|go:"GO:0043066"(negative regulation of apoptotic process)|go:"GO:0051966"(regulation of synaptic transmission, glutamatergic)|go:"GO:0043086"(negative regulation of catalytic activity)|go:"GO:0043524"(negative regulation of neuron apoptotic process)|go:"GO:0055037"(recycling endosome)|go:"GO:0061024"(membrane organization)|go:"GO:0061909"(autophagosome-lysosome fusion)|go:"GO:0044754"(autolysosome)|go:"GO:0070613"(regulation of protein processing)|go:"GO:0072659"(protein localization to plasma membrane)|go:"GO:0044857"(plasma membrane raft organization)|go:"GO:0045121"(membrane raft)|go:"GO:0090160"(Golgi to lysosome transport)|go:"GO:0045861"(negative regulation of proteolysis)|go:"GO:0046474"(glycerophospholipid biosynthetic process)|go:"GO:0046836"(glycolipid transport)|go:"GO:0047496"(vesicle transport along microtubule)|go:"GO:0090384"(phagosome-lysosome docking)|go:"GO:0090385"(phagosome-lysosome fusion)|go:"GO:0048172"(regulation of short-term neuronal synaptic plasticity)|go:"GO:0048306"(calcium-dependent protein binding)|go:"GO:0097352"(autophagosome maturation)|go:"GO:0120146"(sulfatide binding)|go:"GO:1900079"(regulation of arginine biosynthetic process)|go:"GO:1901096"(regulation of autophagosome maturation)|go:"GO:1903076"(regulation of protein localization to plasma membrane)|go:"GO:1905146"(lysosomal protein catabolic process)|go:"GO:1905162"(regulation of phagosome maturation)|go:"GO:1905244"(regulation of modification of synaptic structure)|go:"GO:2001288"(positive regulation of caveolin-mediated endocytosis)|interpro:IPR003492(Batten's disease protein Cln3)|interpro:IPR018460|interpro:IPR036259|mint:Q13286|go:"GO:0106049"(regulation of cellular response to osmotic stress)|refseq:NP_001273033.1|refseq:NP_001273034.1|refseq:NP_000077.1|refseq:NP_001035897.1	ensembl:ENSG00000102805(gene)|ensembl:ENST00000377453(transcript)|ensembl:ENST00000636183(transcript)|go:"GO:0005537"(mannose binding)|go:"GO:0005764"(lysosome)|go:"GO:0005765"(lysosomal membrane)|go:"GO:0005783"(endoplasmic reticulum)|go:"GO:0005794"(Golgi apparatus)|go:"GO:0005829"(cytosol)|go:"GO:0006465"(signal peptide processing)|go:"GO:0007040"(lysosome organization)|go:"GO:0007042"(lysosomal lumen acidification)|go:"GO:0007420"(brain development)|go:"GO:0016021"(integral component of membrane)|go:"GO:0016798"(hydrolase activity, acting on glycosyl bonds)|go:"GO:0030163"(protein catabolic process)|go:"GO:0042147"(retrograde transport, endosome to Golgi)|go:"GO:0042551"(neuron maturation)|go:"GO:0048471"(perinuclear region of cytoplasm)|go:"GO:0070062"(extracellular exosome)|go:"GO:0070085"(glycosylation)|go:"GO:1904426"(positive regulation of GTP binding)|interpro:IPR026138|go:"GO:0022008"(neurogenesis)|refseq:NP_006484.1	-	-	-	figure legend:Fig. 5c|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:-1(in vitro)|taxid:-1(In vitro)	-	2019/12/13	2019/12/16	rogid:zf0wXU6yZF+HuwwuMueb9vKIMy89606	rogid:YBJqxznEkJjC7Rdm95cpHHBPuE49606	rigid:D7pYRB0e7TTsbre3YuwtkbRs91I	false	radiolabel:?-?	glutathione s tranferase tag:?-?	-	-	psi-mi:"MI:0833"(autoradiography)	psi-mi:"MI:0833"(autoradiography)