BioPAX pathway converted from "NDUF subunits bind to form the IP subcomplex" in the Reactome database.LEFT-TO-RIGHTNDUF subunits bind to form the IP subcomplexThe subunits NDUFS7, S8 and A9, together with NDUFS2 and S3, form an evolutionarily conserved hydrogenase module as part of the Iron-Sulfur protein fraction (IP) subcomplex (Mckenzie & Ryan 2010, Andrews et al. 2013).Authored: Jassal, Bijay, 2015-09-30Reviewed: Meldal, Birgit, 2015-10-01Edited: Jassal, Bijay, 2015-09-30NDUFS3NADH-ubiquinone oxidoreductase 30 kDa subunitReactome DB_ID: 163927mitochondrial matrixGENE ONTOLOGYGO:0005759UniProt:O75489 NDUFS3NDUFS3FUNCTION Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis (PubMed:12611891). Complex I functions in the transfer of electrons from NADH to the respiratory chain (By similarity). The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity).SUBUNIT Mammalian complex I is composed of 45 different subunits (PubMed:12611891). Interacts with NDUFAF3 (PubMed:19463981). Interacts with RAB5IF (PubMed:31536960).SIMILARITY Belongs to the complex I 30 kDa subunit family.Homo sapiensNCBI Taxonomy9606UniProtO7548937EQUAL264EQUALReactome Database ID Release 75163927Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=163927ReactomeR-HSA-1639272Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-163927.2Reactomehttp://www.reactome.orgNDUFS7:4Fe-4SComplex I - 20 kDa subunit-4Fe-4S cluster complexReactome DB_ID: 1642934Fe-4S4Iron-4Sulfur ClusterReactome DB_ID: 169274Reactome Database ID Release 75169274Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=169274ReactomeR-ALL-1692742Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-169274.2ChEBI30408additional informationMIMI:03611NDUFS7NADH-ubiquinone oxidoreductase 20 kDa subunitReactome DB_ID: 6788521UniProt:O75251 NDUFS7NDUFS7FUNCTION Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.SUBUNIT Complex I is composed of 45 different subunits This is a component of the iron-sulfur (IP) fragment of the enzyme.PTM Hydroxylated ar Arg-111 by NDUFAF5 early in the pathway of assembly of complex I, before the formation of the juncture between peripheral and membrane arms.SIMILARITY Belongs to the complex I 20 kDa subunit family.UniProtO7525139EQUAL213EQUALReactome Database ID Release 756788521Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6788521ReactomeR-HSA-67885211Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6788521.11Reactome Database ID Release 75164293Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=164293ReactomeR-HSA-1642932Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-164293.2NDUFA9:FADComplex I - 39 kDa subunit-FAD cofactorReactome DB_ID: 164289FADFlavin adenine dinucleotideReactome DB_ID: 113596FAD [ChEBI:16238]FADFlavin adenine dinucleotideChEBICHEBI:16238Reactome Database ID Release 75113596Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=113596ReactomeR-ALL-1135962Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-113596.2COMPOUNDC000161NDUFA9NADH-ubiquinone oxidoreductase 39 kDa subunitReactome DB_ID: 163942UniProt:Q16795 NDUFA9NDUFA9NDUFS2LFUNCTION Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Required for proper complex I assembly (PubMed:28671271). Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.SUBUNIT Complex I is composed of 45 different subunits. This a component of the hydrophobic protein fraction (PubMed:12611891, PubMed:27626371). Interacts with BLOC1S1 (PubMed:22309213). Interacts with SLC2A4 (By similarity). Interacts with CLOCK (PubMed:28985504). Interacts with RAB5IF (PubMed:31536960).PTM Acetylated on lysine residues. BLOC1S1 is required for acetylation (PubMed:22309213). Acetylated by CLOCK in a circadian manner (PubMed:28985504).SIMILARITY Belongs to the complex I NDUFA9 subunit family.UniProtQ1679536EQUAL377EQUALReactome Database ID Release 75163942Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=163942ReactomeR-HSA-1639422Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-163942.21Reactome Database ID Release 75164289Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=164289ReactomeR-HSA-1642892Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-164289.2NDUFAF7:NDUFS2:2x4Fe-4SReactome DB_ID: 164288NDUFAF7NADH dehydrogenase [ubiquinone] complex I, assembly factor 7NDUF7_HUMANReactome DB_ID: 6788525UniProt:Q7L592 NDUFAF7NDUFAF7C2orf56PRO1853FUNCTION Arginine methyltransferase involved in the assembly or stability of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I) (PubMed:20406883, PubMed:24089531, PubMed:24838397). Acts by mediating symmetric dimethylation of 'Arg-118' of NDUFS2 after it assembles into the complex I, stabilizing the early intermediate complex (PubMed:24089531).SUBUNIT Interacts with NDUFS2 (PubMed:20406883, PubMed:24089531).DISEASE Defects in NDUFAF7 may be a cause of susceptibility to pathologic myopia, a genetically heterogeneous disorder characterized by extreme, familial, early-onset vision loss and described as myopia accompanied by severe deformation of the eye besides excessive elongation of the eye.SIMILARITY Belongs to the NDUFAF7 family.CAUTION Stoichiometry of the protein is unclear. According to a report, it forms a homodimer (PubMed:20406883). According to another publication, it is mainly monomeric (PubMed:24838397).UniProtQ7L59247EQUAL441EQUALReactome Database ID Release 756788525Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6788525ReactomeR-HSA-67885251Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6788525.11NDUFS2:4Fe-4SReactome DB_ID: 5690027NDUFS2NADH-ubiquinone oxidoreductase 49 kDa subunitReactome DB_ID: 6788520UniProt:O75306 NDUFS2NDUFS2FUNCTION Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (PubMed:12611891).SUBUNIT Complex I is composed of 45 different subunits. Component of the iron-sulfur (IP) fragment of the enzyme (PubMed:12611891). Interacts with NDUFAF3 (PubMed:19463981). Interacts with NDUFAF7 (PubMed:20406883, PubMed:24089531).PTM Dimethylation at Arg-118 by NDUFAF7 takes place after NDUFS2 assembles into the complex I, leading to stabilize the early intermediate complex (PubMed:24089531, PubMed:24838397).SIMILARITY Belongs to the complex I 49 kDa subunit family.UniProtO7530634EQUAL463EQUALReactome Database ID Release 756788520Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6788520ReactomeR-HSA-67885201Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6788520.111Reactome Database ID Release 755690027Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=5690027ReactomeR-HSA-56900272Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5690027.21Reactome Database ID Release 75164288Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=164288ReactomeR-HSA-1642881Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-164288.1NDUFS8:2x4Fe-4SComplex I - 23 kDa subunit-2 x 4Fe-4S cluster complexReactome DB_ID: 164295NDUFS8NADH-ubiquinone oxidoreductase 23 kDa subunitReactome DB_ID: 6788526UniProt:O00217 NDUFS8NDUFS8FUNCTION Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.SUBUNIT Mammalian complex I is composed of 45 different subunits (PubMed:12611891). This is a component of the iron-sulfur (IP) fragment of the enzyme (PubMed:12611891). Interacts with RAB5IF (PubMed:31536960).TISSUE SPECIFICITY Expressed in all tissues with the highest level in heart and skeletal muscle and the lowest level in lung.SIMILARITY Belongs to the complex I 23 kDa subunit family.UniProtO0021735EQUAL210EQUALReactome Database ID Release 756788526Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6788526ReactomeR-HSA-67885261Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6788526.112Reactome Database ID Release 75164295Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=164295ReactomeR-HSA-1642952Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-164295.2IP subcomplexIron-Sulfur Protein fractionReactome DB_ID: 568969911111Reactome Database ID Release 755689699Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=5689699ReactomeR-HSA-56896993Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5689699.3Reactome Database ID Release 756800868Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=6800868ReactomeR-HSA-68008681Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6800868.120552642Pubmed2010Assembly factors of human mitochondrial complex I and their defects in diseaseMcKenzie, MatthewRyan, Michael TIUBMB Life 62:497-50224191001Pubmed2013Assembly factors for the membrane arm of human complex IAndrews, ByronCarroll, JoeDing, ShujingFearnley, Ian MWalker, JEProc. Natl. Acad. Sci. U.S.A. 110:18934-9