#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:P36776-1	uniprotkb:P36776-1	intact:EBI-25473602|ensembl:ENSP00000353826	intact:EBI-25473602|ensembl:ENSP00000353826	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:"MI:0007"(anti tag coimmunoprecipitation)	Strauss et al. (2015)	pubmed:25574826|imex:IM-27801	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-25473609|imex:IM-27801-1	-	-	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:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	ensembl:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	-	-	-	figure legend:4e|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:9606(human-293t)|taxid:9606(Homo sapiens 293 cells transformed with SV40 large T antigen)	-	2020/03/17	2020/03/20	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rigid:nLjkxxDRrq1JCwGQTUfqAv6byl4	false	v5 tag:c-c|mutation with no effect:721-721(rs147588238)	flag tag:c-c	-	-	psi-mi:"MI:0705"(anti tag western blot)	psi-mi:"MI:0705"(anti tag western blot)
uniprotkb:P36776-1	uniprotkb:P36776-1	intact:EBI-25473602|ensembl:ENSP00000353826	intact:EBI-25473602|ensembl:ENSP00000353826	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:"MI:0007"(anti tag coimmunoprecipitation)	Strauss et al. (2015)	pubmed:25574826|imex:IM-27801	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-25473620|imex:IM-27801-3	-	-	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:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	ensembl:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	-	-	-	figure legend:4e|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:9606(human-293t)|taxid:9606(Homo sapiens 293 cells transformed with SV40 large T antigen)	-	2020/03/17	2020/03/20	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rigid:nLjkxxDRrq1JCwGQTUfqAv6byl4	false	v5 tag:c-c	flag tag:c-c|mutation decreasing interaction strength:721-721(rs147588238)	-	-	psi-mi:"MI:0705"(anti tag western blot)	psi-mi:"MI:0705"(anti tag western blot)
uniprotkb:P36776-1	uniprotkb:P36776-1	intact:EBI-25473602|ensembl:ENSP00000353826	intact:EBI-25473602|ensembl:ENSP00000353826	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:"MI:0007"(anti tag coimmunoprecipitation)	Strauss et al. (2015)	pubmed:25574826|imex:IM-27801	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-25473634|imex:IM-27801-5	-	-	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:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	ensembl:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	intact:EBI-25473609(see-also)|intact:EBI-25473620(see-also)	-	-	figure legend:4e|comment:Co-immunoprecipitation of p.Arg721-Gly V5 by p.Arg721Gly FLAG was barely detected, suggesting impaired homo-oligomeric assembly of p.Arg721Gly or disassembly of an unstable complex during the experimental procedure.|dataset:Rare diseases - Interactions investigated in the context of Rare genetic disease|full coverage:Only protein-protein interactions|curation depth:imex curation	taxid:9606(human-293t)|taxid:9606(Homo sapiens 293 cells transformed with SV40 large T antigen)	-	2020/03/17	2020/03/20	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rigid:nLjkxxDRrq1JCwGQTUfqAv6byl4	false	v5 tag:c-c|mutation decreasing interaction strength:721-721(rs147588238)	flag tag:c-c|mutation decreasing interaction strength:721-721(rs147588238)	-	-	psi-mi:"MI:0705"(anti tag western blot)	psi-mi:"MI:0705"(anti tag western blot)
uniprotkb:P51557	uniprotkb:P36776-1	intact:EBI-9817935|uniprotkb:Q543A5|uniprotkb:Q924Y5|uniprotkb:Q9D2G1|ensembl:ENSMUSP00000033979	intact:EBI-25473602|ensembl:ENSP00000353826	psi-mi:star_mouse(display_long)|uniprotkb:Star(gene name)|psi-mi:Star(display_short)|uniprotkb:Luteinizing hormone-induced protein(gene name synonym)|uniprotkb:START domain-containing protein 1(gene name synonym)	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Strauss et al. (2015)	pubmed:25574826|imex:IM-27801	taxid:10090(mouse)|taxid:10090(Mus musculus)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-25473695|imex:IM-27801-9	-	-	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	refseq:NP_035615.2|ensembl:ENSMUSG00000031574(gene)|ensembl:ENSMUST00000033979(transcript)|go:"GO:0005739"(mitochondrion)|go:"GO:0005758"(mitochondrial intermembrane space)|go:"GO:0005829"(cytosol)|go:"GO:0006694"(steroid biosynthetic process)|go:"GO:0006699"(bile acid biosynthetic process)|go:"GO:0006703"(estrogen biosynthetic process)|go:"GO:0007420"(brain development)|go:"GO:0007584"(response to nutrient)|go:"GO:0008203"(cholesterol metabolic process)|go:"GO:0008211"(glucocorticoid metabolic process)|go:"GO:0008584"(male gonad development)|go:"GO:0009635"(response to herbicide)|go:"GO:0010212"(response to ionizing radiation)|go:"GO:0010288"(response to lead ion)|go:"GO:0010628"(positive regulation of gene expression)|go:"GO:0014823"(response to activity)|go:"GO:0015485"(cholesterol binding)|go:"GO:0016101"(diterpenoid metabolic process)|go:"GO:0017143"(insecticide metabolic process)|go:"GO:0018879"(biphenyl metabolic process)|go:"GO:0018894"(dibenzo-p-dioxin metabolic process)|go:"GO:0018958"(phenol-containing compound metabolic process)|go:"GO:0018963"(phthalate metabolic process)|go:"GO:0030061"(mitochondrial crista)|go:"GO:0032367"(intracellular cholesterol transport)|go:"GO:0032869"(cellular response to insulin stimulus)|go:"GO:0035094"(response to nicotine)|go:"GO:0035457"(cellular response to interferon-alpha)|go:"GO:0042493"|go:"GO:0042542"(response to hydrogen peroxide)|go:"GO:0042747"(circadian sleep/wake cycle, REM sleep)|go:"GO:0043005"(neuron projection)|go:"GO:0043025"(neuronal cell body)|go:"GO:0043524"(negative regulation of neuron apoptotic process)|go:"GO:0043627"(response to estrogen)|go:"GO:0044255"(cellular lipid metabolic process)|go:"GO:0044321"(response to leptin)|go:"GO:0044344"(cellular response to fibroblast growth factor stimulus)|go:"GO:0045471"(response to ethanol)|go:"GO:0048168"(regulation of neuronal synaptic plasticity)|go:"GO:0050769"(positive regulation of neurogenesis)|go:"GO:0050810"(regulation of steroid biosynthetic process)|go:"GO:0051412"(response to corticosterone)|go:"GO:0060992"(response to fungicide)|go:"GO:0061370"(testosterone biosynthetic process)|go:"GO:0070859"(positive regulation of bile acid biosynthetic process)|go:"GO:0071222"(cellular response to lipopolysaccharide)|go:"GO:0071236"(cellular response to antibiotic)|go:"GO:0071276"(cellular response to cadmium ion)|go:"GO:0071312"(cellular response to alkaloid)|go:"GO:0071320"(cellular response to cAMP)|go:"GO:0071333"(cellular response to glucose stimulus)|go:"GO:0071346"(cellular response to interferon-gamma)|go:"GO:0071372"(cellular response to follicle-stimulating hormone stimulus)|go:"GO:0071373"(cellular response to luteinizing hormone stimulus)|go:"GO:0071378"(cellular response to growth hormone stimulus)|go:"GO:0071549"(cellular response to dexamethasone stimulus)|go:"GO:0071560"(cellular response to transforming growth factor beta stimulus)|go:"GO:0071872"(cellular response to epinephrine stimulus)|go:"GO:0120020"(cholesterol transfer activity)|interpro:IPR000799(Steroidogenic acute regulatory protein)|interpro:IPR002913(Lipid-binding START)|interpro:IPR023393|interpro:IPR029866|reactome:R-MMU-196108	ensembl:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	go:"GO:0004176"(ATP-dependent peptidase activity)	-	-	figure legend:4b, S1a|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)	-	2020/03/17	2020/04/27	rogid:T2DTbpy1uwAX3W6xHt6UIozuYlk10090	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rigid:dcFaibciM9qyHknZA1foTfwX+wI	false	-	his tag:n-n|mutation decreasing interaction rate:676-676(rs879255247)|mutation decreasing interaction rate:721-721(rs147588238)|sufficient binding region:115-959	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:Q00059	uniprotkb:P36776-1	intact:EBI-1049924|uniprotkb:Q5U0C6|uniprotkb:B5BU05|uniprotkb:A9QXC6|uniprotkb:A8MRB2|ensembl:ENSP00000420588	intact:EBI-25473602|ensembl:ENSP00000353826	psi-mi:tfam_human(display_long)|uniprotkb:TCF6(gene name synonym)|uniprotkb:Mitochondrial transcription factor 1(gene name synonym)|uniprotkb:Transcription factor 6-like 2(gene name synonym)|uniprotkb:TFAM(gene name)|psi-mi:TFAM(display_short)|uniprotkb:TCF6L2(gene name synonym)|uniprotkb:Transcription factor 6(gene name synonym)	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:"MI:0435"(protease assay)	Strauss et al. (2015)	pubmed:25574826|imex:IM-27801	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:9606(human)|taxid:9606(Homo sapiens)	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-25474221|imex:IM-27801-11	-	-	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0326"(protein)	refseq:NP_003192.1|refseq:NP_001257711.1|go:"GO:0034246"(mitochondrial transcription factor activity)|go:"GO:0042645"(mitochondrial nucleoid)|go:"GO:0043565"(sequence-specific DNA binding)|go:"GO:0045893"(positive regulation of transcription, DNA-templated)|interpro:IPR009071(High mobility group box, HMG)|interpro:IPR036910|mint:Q00059|rcsb pdb:3FGH|rcsb pdb:3TMM|rcsb pdb:3TQ6|rcsb pdb:4NNU|rcsb pdb:4NOD|rcsb pdb:6ERP|rcsb pdb:6ERQ|rcsb pdb:6HB4|rcsb pdb:6HC3|go:"GO:0033108"(mitochondrial respiratory chain complex assembly)|reactome:R-HSA-163282|reactome:R-HSA-2151201|go:"GO:0032991"(protein-containing complex)|ensembl:ENSG00000108064(gene)|ensembl:ENST00000487519(transcript)|go:"GO:0000976"(transcription cis-regulatory region binding)|go:"GO:0001018"(mitochondrial promoter sequence-specific DNA binding)|go:"GO:0001223"(transcription coactivator binding)|go:"GO:0003682"(chromatin binding)|go:"GO:0003723"(RNA binding)|go:"GO:0005634"(nucleus)|go:"GO:0005739"(mitochondrion)|go:"GO:0005759"(mitochondrial matrix)|go:"GO:0005829"(cytosol)|go:"GO:0006390"(mitochondrial transcription)|go:"GO:0006391"(transcription initiation from mitochondrial promoter)|go:"GO:0008301"(DNA binding, bending)|go:"GO:0031072"(heat shock protein binding)	ensembl:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	go:"GO:0004176"(ATP-dependent peptidase activity)	-	-	figure legend:4b, S1b, S1c|comment:"There is discrepancy on the positioning of the His tag in TRAM and LONP1 between the methods and in the figure S1 legend; n-terminal position has been adopted after checking for the LONP1 vector (proEx-1) and also checking back referenced 23201127"|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)	-	2020/03/17	2020/04/27	rogid:JqG4yJbWWsYMvex7a6GDIDxiVcs9606	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rigid:XJ1hL7bjMzO5MQmtu6GChAAlRso	false	his tag:n-n	his tag:n-n|mutation with no effect:676-676(rs879255247)|mutation with no effect:721-721(rs147588238)|sufficient binding region:115-959	-	-	psi-mi:"MI:0113"(western blot)	psi-mi:"MI:0113"(western blot)
uniprotkb:P36776-1	intact:EBI-25473661	intact:EBI-25473602|ensembl:ENSP00000353826	-	psi-mi:p36776-1(display_long)|uniprotkb:LONP1(gene name)|psi-mi:LONP1(display_short)|uniprotkb:PRSS15(gene name synonym)|uniprotkb:LONHs(gene name synonym)|uniprotkb:Lon protease-like protein(gene name synonym)|uniprotkb:Mitochondrial ATP-dependent protease Lon(gene name synonym)|uniprotkb:Serine protease 15(gene name synonym)	psi-mi:s2 peptide(display_short)|psi-mi:EBI-25473661(display_long)	psi-mi:"MI:0435"(protease assay)	Strauss et al. (2015)	pubmed:25574826|imex:IM-27801	taxid:9606(human)|taxid:9606(Homo sapiens)	taxid:-2(chemical synthesis)|taxid:-2("Chemical synthesis (Chemical synthesis)")	psi-mi:"MI:0570"(protein cleavage)	psi-mi:"MI:0469"(IntAct)	intact:EBI-25473663|imex:IM-27801-7	-	-	psi-mi:"MI:0501"(enzyme)	psi-mi:"MI:0502"(enzyme target)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0497"(neutral component)	psi-mi:"MI:0326"(protein)	psi-mi:"MI:0327"(peptide)	ensembl:ENSG00000196365(gene)|ensembl:ENST00000360614(transcript)|intact:EBI-357448(isoform-parent)	-	-	-	-	figure legend:4a|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)	-	2020/03/17	2020/04/28	rogid:quAPz1dZK2XdoE6WMjQJwFtx53o9606	rogid:a4QNEm4CVDHqmVvAL+uBxxq07IA-2	rigid:OZhNh/+MnzG30av9xxraOMmu6gI	false	sufficient binding region:115-959|his tag:n-n|mutation decreasing interaction rate:721-721(rs147588238)|mutation decreasing interaction rate:631-631(rs879255248)|mutation decreasing interaction rate:676-676(rs879255247)|mutation decreasing interaction rate:724-724(rs879255249)	fluorophore:c-c	-	-	psi-mi:"MI:0867"(tag visualisation by fluorescence)	psi-mi:"MI:0867"(tag visualisation by fluorescence)