Mitochondrial complex I assembly model OXPHOS system (Homo sapiens)

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MT-ND5NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1DMAC117NDUFAF29, 12, 17NDUFS31171711, 17711, 1711, 1714112027, 289, 12, 171713111121717, 25COMPLEX 1Additional subunitsfor final complex formationIMSComplex I assembly:EC 1.6.5.3+ND4-moduleTimeline of incorporationCHAPERONESND2-moduleQ/ND1-moduleQ-moduleN-moduleNDUFA6NDUFAF3TMEM186FOXRED1MT-ND5MT-ND4MT-ND3DMAC1MT-ND1NDUFA3NDUFB5Electron Transport Chain PWTMEM126BNDUFV1NDUFAF2NDUFS1COA1[4Fe-4A]clustersNDUFA5NDUFA1TIMMDC19, 12, 1711, 18, 2411C10, 15173, 22, 23, 27CNDUFV2CCNDUFA2NDUFV1NDUFV2NDUFS1NDUFA2NDUFA7NDUFA12NDUFS4NDUFS6NDUFV3NDUFB10NDUFB11NDUFB6NDUFB5NDUFB10NDUFB11NDUFB6NDUFB1NDUFB4NDUFB5NDUFB10NDUFB11NDUFB6MT-ND4NDUFB1NDUFB4ATP5SLTMEM7010, 1711, 22, 26MTND2NDUFC1NDUFC2NDUFAF16, 19ECSITACAD9295, 21MT-ND3MT-ND6MT-ND4LMT-ND6MT-ND4LMTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9MT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9COA1COA1C11, 18, 2418, 24CTMEM186CCOA111CNDUFA1018, 24NDUFS5MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS511, 17TMEM186COA12TMEM126BNDUFAF16, 19ECSITACAD9295, 21NDUFAF6NUBPL16NDUFS2NDUFAF7[4Fe-4A]clustersNUBPL27, 28[4Fe-4A]clustersNDUFS2NDUFS8MMNDUFAF58NDUFS7COOHNDUFS7NDUFAF44Cnecessary to maintain normal MT-ND1 synthesis[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS8NDUFS78NDUFAF6NDUFAF316NDUFAF4CC411NDUFA8NDUFA13[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS8NDUFS78NDUFAF6NDUFAF316CNDUFAF4CMT-ND14TIMMDC110, 15C[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS8NDUFS78NDUFAF6NDUFAF316NDUFAF4C4CMT-ND1TIMMDC110, 15CNDUFA3NDUFA8NDUFA13NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1ND5-moduleMT-ND5NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1DMAC117MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5COA1COA111, 18, 24TMEM18611, 18, 2411COA1TMEM18618, 2411COA118, 24FOXRED13, 22, 23, 27ATP5SL10, 17TMEM70TMEM18611, 22, 26C11COA1C18, 24Mitochondrial Complex I Assembly (MCIA) complex2, 15MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5FOXRED1ATP5SL3, 22, 23, 2710, 17TMEM70TMEM18611, 22, 2611COA1C18, 24C[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS88NDUFS7NDUFAF616NDUFAF3NDUFAF4C4CMT-ND1TIMMDC1CNDUFA310, 15NDUFA8NDUFA13MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5FOXRED1ATP5SL3, 22, 23, 2710, 17TMEM70TMEM18611, 22, 2611CCOA1C18, 24[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS8NDUFS78NDUFAF6NDUFAF316CNDUFAF4MT-ND1C4TIMMDC1NDUFA310, 15CNDUFA8NDUFA13MT-ND5NDUFB2NDUFB3NDUFB7NDUFB8NDUFB9NDUFAB1DMAC1NDUFAF2179, 12, 17MT-ND6MT-ND4LMT-ND3MTND2NDUFC1NDUFC2NDUFAF1ECSITACAD9NDUFA1NDUFA10NDUFS5FOXRED1ATP5SL3, 22, 23, 27TMEM7010, 1711, 22, 26TMEM186CCOA11118, 24C[4Fe-4A]clustersNDUFS3NDUFA5NDUFS2NDUFS88NDUFS7NDUFAF616NDUFAF3NDUFAF4C4CMT-ND1TIMMDC110, 15CNDUFA3NDUFA8NDUFA13NDUFA6NDUFA7NDUFA12NDUFS4NDUFS6NDUFV3NDUFV1NDUFV2NDUFS1NDUFA2IMMatrixTMEM126B