IntAct European Bioinformatics Institute IntAct intact http://www.ebi.ac.uk/ http://www.ebi.ac.uk/intact/query/${ac} EBI-[0-9]+|IA:[0-9]+ INTerAction database (IntAct) provides an open source database and toolkit for the storage, presentation and analysis of molecular interactions. http://www.ebi.ac.uk/intact European Bioinformatics Institute; Wellcome Trust Genome Campus; Hinxton, Cambridge; CB10 1SD; United Kingdom http://www.ebi.ac.uk/intact/ Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance. Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance. Biochimica et biophysica acta. Proteins and proteomics 2020 imex curation Ribeiro JV., Lucas TG., Bross P., Gomes CM., Henriques BJ. Rare diseases - Interactions investigated in the context of Rare genetic disease bjhenriques@fc.ul.pt 28-Sep-2020: Contacted by IntAct-Help. Only protein-protein interactions imex curation in vitro In vitro uv-vis ultraviolet-visible spectroscopy uv-vis predetermined predetermined participant predetermined Ribeiro JV., Lucas TG., Bross P., Gomes CM., Henriques BJ. Rare diseases - Interactions investigated in the context of Rare genetic disease bjhenriques@fc.ul.pt Accepted 2020-SEP-24 AT 07:05 BST AT 07:05 BST by ORCHARD Biochimica et biophysica acta. Proteins and proteomics 2020 Only protein-protein interactions imex curation imex curation Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance. Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance. Biochimica et biophysica acta. Proteins and proteomics 2020 imex curation Ribeiro JV., Lucas TG., Bross P., Gomes CM., Henriques BJ. Rare diseases - Interactions investigated in the context of Rare genetic disease bjhenriques@fc.ul.pt 28-Sep-2020: Contacted by IntAct-Help. Only protein-protein interactions imex curation in vitro In vitro oxidoreduct assay oxidoreductase assay oxidoreduct assay predetermined predetermined participant predetermined Ribeiro JV., Lucas TG., Bross P., Gomes CM., Henriques BJ. Rare diseases - Interactions investigated in the context of Rare genetic disease 2,6-dichloroindophenol (CHEBI:945) acts as electron donor bjhenriques@fc.ul.pt Accepted 2020-SEP-24 AT 07:05 BST AT 07:05 BST by ORCHARD Biochimica et biophysica acta. Proteins and proteomics 2020 Only protein-protein interactions imex curation imex curation 5-methylphenazinium methyl sulfate 5-methylphenazinium methyl sulfate 5-Methyl phenazine, methyl sulfate salt 5-Methylphenazine methylsulfate 5-Methylphenazinium methyl sulphate Methylphenazonium methosulfate N-Methylphenazonium methosulfate N-Methylphenazonium methosulphate Phenazine methosulfate Phenazine methosulphate PMS 5-methylphenazin-5-ium methyl sulfate small molecule small molecule chemical synthesis Chemical synthesis (Chemical synthesis) gcdh_human Glutaryl-CoA dehydrogenase, mitochondrial GCDH protein protein human Homo sapiens Human MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK 415B8D510027BB63 glutaryl-coa glutaryl-CoA 4-carboxybutanoyl-coenzyme A coenzyme A, S-(hydrogen pentanedioate) Glutaryl-CoA glutaryl-coenzyme A 3'-phosphoadenosine 5'-{3-[(3R)-4-{[3-({2-[(4-carboxybutanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-3-hydroxy-2,2-dimethyl-4-oxobutyl] dihydrogen diphosphate} 4-carboxybutanoyl-CoA small molecule small molecule chemical synthesis Chemical synthesis (Chemical synthesis) etfd_human Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial ETFDH Electron-transferring-flavoprotein dehydrogenase protein protein human Homo sapiens Human MLVPLAKLSCLAYQCFHALKIKKNYLPLCATRWSSTSTVPRITTHYTIYPRDKDKRWEGVNMERFAEEADVVIVGAGPAGLSAAVRLKQLAVAHEKDIRVCLVEKAAQIGAHTLSGACLDPGAFKELFPDWKEKGAPLNTPVTEDRFGILTEKYRIPVPILPGLPMNNHGNYIVRLGHLVSWMGEQAEALGVEVYPGYAAAEVLFHDDGSVKGIATNDVGIQKDGAPKATFERGLELHAKVTIFAEGCHGHLAKQLYKKFDLRANCEPQTYGIGLKELWVIDEKNWKPGRVDHTVGWPLDRHTYGGSFLYHLNEGEPLVALGLVVGLDYQNPYLSPFREFQRWKHHPSIRPTLEGGKRIAYGARALNEGGFQSIPKLTFPGGLLIGCSPGFMNVPKIKGTHTAMKSGILAAESIFNQLTSENLQSKTIGLHVTEYEDNLKNSWVWKELYSVRNIRPSCHGVLGVYGGMIYTGIFYWILRGMEPWTLKHKGSDFERLKPAKDCTPIEYPKPDGQISFDLLSSVALSGTNHEHDQPAHLTLRDDSIPVNRNLSIYDGPEQRFCPAGVYEFVPVEQGDGFRLQINAQNCVHCKTCDIKDPSQNINWVVPEGGGGPAYNGM 099EBA36C59AF3D6 fad FAD - Flavin adenine dinucleotide Adenosine 5'-(trihydrogen pyrophosphate), 5'-5'-ester with riboflavine adenosine 5'-[3-(riboflavin-5'-yl) dihydrogen diphosphate] FAD Flavin adenine dinucleotide FLAVIN-ADENINE DINUCLEOTIDE Riboflavin 5'-(trihydrogen diphosphate), 5'-5'-ester with adenosine Riboflavin 5'-adenosine diphosphate adenosine 5'-(3-{D-ribo-5-[7,8-dimethyl-2,4-dioxo-3,4-dihydrobenzo[g]pteridin-10(2H)-yl]-2,3,4-trihydroxypentyl} dihydrogen diphosphate) small molecule small molecule chemical synthesis Chemical synthesis (Chemical synthesis) Flavin adenine dinucleotide Flavin adenine dinucleotide fad-gcdh-1 1 4 unspecified role unspecified role neutral component neutral component purified purified Q92947:p.Arg227Pro mutation with no effect mutation with no effect mutation with no effect mutation not affecting interaction certain certain sequence position certain certain certain sequence position certain R P Q92947:p.Val400Met mutation decreasing strength mutation decreasing interaction strength mutation decreasing strength certain certain sequence position certain certain certain sequence position certain V M c-terminus his tag his tag Hexa-His-tag 6-His-tag Histidine-tag Hexa-His-tag Histidine-tag 6-His-tag c-term range C-terminal range c-term range c-term range C-terminal range c-term range ecoli Escherichia coli (strain K12) 7 unspecified role unspecified role neutral component neutral component purified purified chemical synthesis Chemical synthesis (Chemical synthesis) direct interaction direct interaction Fig. 4 5_methylphenazinium methyl sulfate-gcdh-1 2 3 enzyme target enzyme target substrate substrate neutral component neutral component purified purified chemical synthesis Chemical synthesis (Chemical synthesis) 4 enzyme enzyme neutral component neutral component purified purified Q92947:p.Arg227Pro mutation decreasing rate mutation decreasing interaction rate mutation decreasing rate certain certain sequence position certain certain certain sequence position certain R P Q92947:p.Val400Met mutation decreasing rate mutation decreasing interaction rate mutation decreasing rate certain certain sequence position certain certain certain sequence position certain V M c-terminus his tag his tag Hexa-His-tag 6-His-tag Histidine-tag Hexa-His-tag Histidine-tag 6-His-tag c-term range C-terminal range c-term range c-term range C-terminal range c-term range redox reaction oxidoreductase activity electron transfer reaction redox reaction Table 2 etfdh-gcdh-1 2 6 enzyme target enzyme target substrate substrate neutral component neutral component purified purified ecoli Escherichia coli (strain K12) 4 enzyme enzyme neutral component neutral component purified purified c-terminus his tag his tag Hexa-His-tag 6-His-tag Histidine-tag Hexa-His-tag Histidine-tag 6-His-tag c-term range C-terminal range c-term range c-term range C-terminal range c-term range Q92947:p.Arg227Pro mutation disrupting rate mutation disrupting interaction rate mutation disrupting rate certain certain sequence position certain certain certain sequence position certain R P Q92947:p.Val400Met mutation decreasing rate mutation decreasing interaction rate mutation decreasing rate certain certain sequence position certain certain certain sequence position certain V M redox reaction oxidoreductase activity electron transfer reaction redox reaction Table 2 etfdh-gcdh-2 2 5 enzyme target enzyme target substrate substrate neutral component neutral component purified purified chemical synthesis Chemical synthesis (Chemical synthesis) 4 enzyme enzyme neutral component neutral component purified purified c-terminus his tag his tag Hexa-His-tag 6-His-tag Histidine-tag Hexa-His-tag Histidine-tag 6-His-tag c-term range C-terminal range c-term range c-term range C-terminal range c-term range Q92947:p.Arg227Pro mutation disrupting rate mutation disrupting interaction rate mutation disrupting rate certain certain sequence position certain certain certain sequence position certain R P Q92947:p.Val400Met mutation decreasing rate mutation decreasing interaction rate mutation decreasing rate certain certain sequence position certain certain certain sequence position certain V M redox reaction oxidoreductase activity electron transfer reaction redox reaction Table 3, Fig. 5